BACK (BTB and C-terminal Kelch) domain found in BTB/POZ domain-containing protein 9 (BTBD9); BTBD9 is a risk factor for Restless Legs Syndrome (RLS) encoding a Cullin-3 substrate adaptor. The BTBD9 gene may be associated with antipsychotic-induced RLS in schizophrenia. Mutations in BTBD9 lead to reduced dopamine, increased locomotion and sleep fragmentation.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529  78 LNIQNVCMTFDVASLYSLPKLTCMCCMFMDRNAQEVLSSEGFLSLSKTALLNIVLRDSFAAPEKDIFLALLNWCKHNS-- 155
Cdd:cd14822    1 LNIRNVCLIYDVASLYSLDDLCQTCCQFMDRNAQEVLSSEGFLTLSKSALKELLSRDSFCAPEVDIFNAVRRWCEHNPds 80

                         90       100
                 ....*....|....*....|.
gi 288915529 156 KENHAEIMQAVRLPLMSLTEL 176
Cdd:cd14822   81 KEDAKEILEAVRLPLMSLTEL 101

F5/8 type C domain; This domain is also known as the discoidin (DS) domain family.

                          10        20        30        40        50        60        70        80
                  ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529  415 NALLNGDTKNYdWdsgytcHQLGSGA---IVVQLAQPYMIGSIRLLLW--DCDDRSYSYYVEVSTNQQQWTMVADRTKVS 489
Cdd:pfam00754  15 AAALDGDPNTA-W------SAWSGDDpqwIQVDLGKPKKITGVVTQGRqdGSNGYVTSYKIEYSLDGENWTTVKDEKIPG 87

                          90       100       110
                  ....*....|....*....|....*....|..
gi 288915529  490 C---KSWQSVTFErQP--ASFIRIVGTHNTAN 516
Cdd:pfam00754  88 NndnNTPVTNTFD-PPikARYVRIVPTSWNGG 118

BACK (BTB and C-terminal Kelch) domain found in BTB/POZ domain-containing protein 9 (BTBD9); BTBD9 is a risk factor for Restless Legs Syndrome (RLS) encoding a Cullin-3 substrate adaptor. The BTBD9 gene may be associated with antipsychotic-induced RLS in schizophrenia. Mutations in BTBD9 lead to reduced dopamine, increased locomotion and sleep fragmentation.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529  78 LNIQNVCMTFDVASLYSLPKLTCMCCMFMDRNAQEVLSSEGFLSLSKTALLNIVLRDSFAAPEKDIFLALLNWCKHNS-- 155
Cdd:cd14822    1 LNIRNVCLIYDVASLYSLDDLCQTCCQFMDRNAQEVLSSEGFLTLSKSALKELLSRDSFCAPEVDIFNAVRRWCEHNPds 80

                         90       100
                 ....*....|....*....|.
gi 288915529 156 KENHAEIMQAVRLPLMSLTEL 176
Cdd:cd14822   81 KEDAKEILEAVRLPLMSLTEL 101

BTB And C-terminal Kelch; This domain is found associated with pfam00651 and pfam01344. The BACK domain is found juxtaposed to the BTB domain; they are separated by as little as two residues. This family appears to be closely related to the BTB domain (Finn RD, personal observation).

                          10        20        30        40        50        60        70        80
                  ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529   83 VCMTFDVASLYSLPKLTCMCCMFMDRNAQEVLSSEGFLSLSKTALLNIVLRDSF-AAPEKDIFLALLNWCKHNS---KEN 158
Cdd:pfam07707   1 CLGIYRFADAYGCTELAEAALRFILQNFLEVAKSEEFLELSLEQLLELLSSDDLnVPSEEEVFEAVIRWVKHDVerrKKH 80

                          90       100
                  ....*....|....*....|...
gi 288915529  159 HAEIMQAVRLPLMSLTELLNVVR 181
Cdd:pfam07707  81 LPELLSAVRLPLLSPQYLLNLVE 103

BTB/POZ domain; The BTB (for BR-C, ttk and bab) or POZ (for Pox virus and Zinc finger) domain is present near the N-terminus of a fraction of zinc finger (pfam00096) proteins and in proteins that contain the pfam01344 motif such as Kelch and a family of pox virus proteins. The BTB/POZ domain mediates homomeric dimerization and in some instances heteromeric dimerization. The structure of the dimerized PLZF BTB/POZ domain has been solved and consists of a tightly intertwined homodimer. The central scaffolding of the protein is made up of a cluster of alpha-helices flanked by short beta-sheets at both the top and bottom of the molecule. POZ domains from several zinc finger proteins have been shown to mediate transcriptional repression and to interact with components of histone deacetylase co-repressor complexes including N-CoR and SMRT. The POZ or BTB domain is also known as BR-C/Ttk or ZiN.

                          10        20        30        40        50        60        70
                  ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*.
gi 288915529    3 RALLYGGMRESqPEAEIPLQDTTAEAFTMLLKYIYTGRAtltdEKEEVLLDFLSLAHKYGFPELEDSTSEYLCTIL 78
Cdd:pfam00651  37 KALFSGQESES-SVSEITLDDVSPEDFEALLEFMYTGKL----ISEENVDDLLAAADKLQIPSLVDKCEEFLIKSL 107

kelch-like protein; Provisional

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529  27 EAFTMLLKYIYTGRATLTDEKEEvllDFLSLAHKYGFPELEDSTSEYLCTILNIQNVCMTFDVASLYSLPKLTCMCCMFM 106
Cdd:PHA03098  57 DSFNEVIKYIYTGKINITSNNVK---DILSIANYLIIDFLINLCINYIIKIIDDNNCIDIYRFSFFYGCKKLYSAAYNYI 133

                         90       100       110       120       130       140       150       160
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529 107 DRNAQEVLSSEGFLSLSKTALLNIvLRDS--FAAPEKDIFLALLNWCKHNSKENHAEImqavrlplmslTELLNVVRPSg 184
Cdd:PHA03098 134 RNNIELIYNDPDFIYLSKNELIKI-LSDDklNVSSEDVVLEIIIKWLTSKKNNKYKDI-----------CLILKVLRIT- 200

                 ....*..
gi 288915529 185 LLSPDAI 191
Cdd:PHA03098 201 FLSEEGI 207

BACK (BTB and C-terminal Kelch) domain found in BTB/POZ domain-containing protein 9 (BTBD9); BTBD9 is a risk factor for Restless Legs Syndrome (RLS) encoding a Cullin-3 substrate adaptor. The BTBD9 gene may be associated with antipsychotic-induced RLS in schizophrenia. Mutations in BTBD9 lead to reduced dopamine, increased locomotion and sleep fragmentation.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529  78 LNIQNVCMTFDVASLYSLPKLTCMCCMFMDRNAQEVLSSEGFLSLSKTALLNIVLRDSFAAPEKDIFLALLNWCKHNS-- 155
Cdd:cd14822    1 LNIRNVCLIYDVASLYSLDDLCQTCCQFMDRNAQEVLSSEGFLTLSKSALKELLSRDSFCAPEVDIFNAVRRWCEHNPds 80

                         90       100
                 ....*....|....*....|.
gi 288915529 156 KENHAEIMQAVRLPLMSLTEL 176
Cdd:cd14822   81 KEDAKEILEAVRLPLMSLTEL 101

BTB And C-terminal Kelch; This domain is found associated with pfam00651 and pfam01344. The BACK domain is found juxtaposed to the BTB domain; they are separated by as little as two residues. This family appears to be closely related to the BTB domain (Finn RD, personal observation).

                          10        20        30        40        50        60        70        80
                  ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529   83 VCMTFDVASLYSLPKLTCMCCMFMDRNAQEVLSSEGFLSLSKTALLNIVLRDSF-AAPEKDIFLALLNWCKHNS---KEN 158
Cdd:pfam07707   1 CLGIYRFADAYGCTELAEAALRFILQNFLEVAKSEEFLELSLEQLLELLSSDDLnVPSEEEVFEAVIRWVKHDVerrKKH 80

                          90       100
                  ....*....|....*....|...
gi 288915529  159 HAEIMQAVRLPLMSLTELLNVVR 181
Cdd:pfam07707  81 LPELLSAVRLPLLSPQYLLNLVE 103

BTB (Broad-Complex, Tramtrack and Bric a brac) /POZ (poxvirus and zinc finger) domain found in Dictyostelium discoideum trishanku and similar proteins; Trishanku is a novel regulator required for normal morphogenesis and cell-type stability in Dictyostelium discoideum. It contains a BTB domain. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*
gi 288915529   3 RALLYGGMRESQpEAEIPLQDTTAEAFTMLLKYIYTGRATLTdekEEVLLDFLSLAHKYGFPELE 67
Cdd:cd18314   33 RSMLTGSMIESN-LKEVTLEDVEPEIFETVLKYMYTGQVTLS---EENVLDLLMLASKYQVPDLE 93

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in zinc finger and BTB domain-containing (ZBTB) proteins, Kelch-like (KLHL) proteins, and similar proteins; This family includes a variety of BTB/POZ domain-containing proteins, such as zinc finger and BTB domain-containing (ZBTB) proteins and Kelch-like (KLHL) proteins. They have diverse functions, such as transcriptional regulation, chromatin remodeling, protein degradation and cytoskeletal regulation. Many BTB/POZ proteins contain one or two additional domains, such as kelch repeats, zinc-finger domains, FYVE (Fab1, YOTB, Vac1, and EEA1) fingers, or ankyrin repeats. These special additional domains or interaction partners provide unique characteristics and functions to BTB/POZ proteins. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50
                 ....*....|....*....|....*....|....*....|....*....|....*....
gi 288915529   3 RALLYGGMRESQpEAEIPLQDTTAEAFTMLLKYIYTGRATLTdekEEVLLDFLSLAHKY 61
Cdd:cd18186   28 RAMFSSGMKESS-SSEIELDDVSPEAFEALLDYIYTGELELS---EENVEELLAAADKL 82

BACK (BTB and C-terminal Kelch) domain found in Kelch-like protein 18 (KLHL18); KLHL18 acts as a substrate-specific adaptor for the Cullin3 E3 ubiquitin-protein ligase complex that regulates mitotic entry and ubiquitylates Aurora-A.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529  75 CTILNIQNVCMTFDVASLYSlpkltcMCCMFMDRNAQEVLSSEGFLSLSKTALLNIVLRDSFAAP-EKDIFLALLNWCKH 153
Cdd:cd18457    4 NNCLGIRQFAETMMCSSLVD------AANKYIQQHFIEVSKSEEFLALSKEEVLEILSRDELNVKsEEQVFEAALAWVKY 77

                         90       100
                 ....*....|....*....|..
gi 288915529 154 ---NSKENHAEIMQAVRLPLMS 172
Cdd:cd18457   78 dreNREEYLPELLSKVRLPLLR 99

BACK (BTB and C-terminal Kelch) domain found in BTB/POZ domain-containing protein 6 (BTBD6); BTBD6, also called lens BTB domain protein, is a BTB-domain-containing Kelch-like protein that is required for proper embryogenesis and plays an essential evolutionarily-conserved role during neuronal development.

                         10        20        30        40        50        60        70
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....
gi 288915529  78 LNIQNVCMTFDVASLYSLPKLTCMCCMFMDRNAQEVLSSEGFLSLSKTALLNIVLRDSFAAPEKDIFLALLNWCKHNSK 156
Cdd:cd18525    1 LEAKNACVLLSQSRLFEEPELTQRCWEVIDAQAELALKSEGFCEIDQQTLEIILTRETLNTKEVVVFEAVLNWAEAECK 79

BACK (BTB and C-terminal Kelch) domain; The BACK domain is found in architectures C-terminal to a BTB domain, in a diverse set of architectures together with Kelch, MATH, and/or TAZ domains. It is involved in interactions with the Cullin3 (Cul3) ubiquitin ligase complex, as well as in homo-oligomerization. Most proteins containing the BACK domain are understood to function as adaptor proteins that play a role in ubiquitination of various substrates.

                         10        20        30        40        50
                 ....*....|....*....|....*....|....*....|....*....|...
gi 288915529  79 NIQNVCMTFDVASLYSLPKLTCMCCMFMDRNAQEVLSSEGFLSLSKTALLNIV 131
Cdd:cd14733    1 DPENCLGILELADLYNLEELKEKALKFILENFEEVSKSEEFLELSVELLLELL 53

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain superfamily; Proteins in this superfamily are characterized by the presence of a common protein-protein interaction motif of about 100 amino acids, known as the BTB/POZ domain. Members include transcription factors, oncogenic proteins, ion channel proteins, and potassium channel tetramerization domain (KCTD) proteins. They have been identified in poxviruses and many eukaryotes, and have diverse functions, such as transcriptional regulation, chromatin remodeling, protein degradation and cytoskeletal regulation. Many BTB/POZ proteins contain one or two additional domains, such as kelch repeats, zinc-finger domains, FYVE (Fab1, YOTB, Vac1, and EEA1) fingers, or ankyrin repeats, among others. These special additional domains or interaction partners provide unique characteristics and functions to BTB/POZ proteins. In ion channel proteins and KCTD proteins, the BTB/POZ domain is also called the tetramerization (T1) domain.

                         10        20        30        40        50
                 ....*....|....*....|....*....|....*....|....*....|....*....
gi 288915529   3 RALLYGGMRESQPeAEIPLQDTTAEAFTMLLKYIYTGRATLtdeKEEVLLDFLSLAHKY 61
Cdd:cd01165   25 RALFRGGFRESGQ-AEINLRDISPEDFRALLEFLYGGKRDL---DASNLLELLEAANFL 79

BACK (BTB and C-terminal Kelch) domain found in Kelch-like protein 3 (KLHL3); KLHL3 serves as a substrate adapter in Cullin3 (Cul3) E3 ubiquitin ligase complexes. It is a component of an E3 ubiquitin ligase complex that regulates blood pressure by targeting With-No-Lysine (WNK) kinases for degradation.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529  90 ASLYSLPKLTCMCCMFMDRNAQEVLSSEGFLSLSKTALLNIVLRDSFA-APEKDIFLALLNWCKHNSK---ENHAEIMQA 165
Cdd:cd18513   13 ADVHTCTELLQQANAYAEQHFPEVMLGEEFLSLSLDQVCSLISSDKLTvSSEEKVFEAVISWIKYDKEarlEHMAKLMEH 92

                         90       100       110
                 ....*....|....*....|....*....|....*.
gi 288915529 166 VRLPLMSLTELLNVVRPSGLLS-----PDAILDAIK 196
Cdd:cd18513   93 VRLPLLPRDYLVQTVEEEALIKnnntcKDFLIEAMK 128

BACK (BTB and C-terminal Kelch) domain found in Kelch-like protein 20 (KLHL20); KLHL20, also termed Kelch-like ECT2-interacting protein (KLEIP), or Kelch-like protein X, is a substrate-specific adaptor of a BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complex involved in interferon response and anterograde Golgi to endosome transport. KLHL20 plays a role in actin assembly at cell-cell contact sites of Madin-Darby canine kidney cells. It also controls endothelial migration and sprouting angiogenesis.

                         10        20        30        40        50        60        70
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|..
gi 288915529 105 FMDRNAQEVLSSEGFLSLSKTALLNIVLRDSF-AAPEKDIFLALLNWCKHNSKENH---AEIMQAVRLPLMS 172
Cdd:cd18459   29 FTQHNFQEVMESEEFMLLPVNQLIDIISSDELnVRSEEQVFNAVMAWVKYNIQERRphlPQVLQHVRLPLLS 100

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in BTB/POZ domain-containing proteins, BTBD3 and BTBD6; This family includes BTB/POZ domain-containing proteins BTBD3 and BTBD6, both of which are BTB-domain-containing Kelch-like proteins. BTBD3 controls dendrite orientation toward active axons in mammalian neocortex. BTBD6 is required for proper embryogenesis and plays an essential evolutionary conserved role during neuronal development. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*
gi 288915529   4 ALLYGGMRESQPEAEIPlqDTTAEAFTMLLKYIYTGRATLTdekEEVLLDFLSLAHKYGFPELEDSTSEYLCTIL 78
Cdd:cd18282   39 AMFYGGLAENKNEIEIP--DVEPAAFLNLLRYLYCDEIDLE---PDTVLATLYAAKKYLVPHLARACVQFLETSL 108

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in Ankyrin repeat and BTB/POZ domain-containing protein 2 (ABTB2) and similar proteins; This family includes ABTB2, BTBD11, plant ARM repeat protein interacting with ABF2 (ARIA), and similar proteins. ABTB2, also called bood POZ containing gene type 2 (BPOZ-2), is a scaffold protein that controls the degradation of many biological proteins ranging from embryonic development to tumor progression. It may be involved in the initiation of hepatocyte growth. ABTB2 functions as an adaptor protein for the E3 ubiquitin ligase scaffold protein Cullin-3. It directly binds to eukaryotic elongation factor 1A1 (eEF1A1) to promote eEF1A1 ubiquitylation and degradation, and prevent translation. The BTBD11 gene has been recently identified as an all-trans retinoic acid (atRA)-responsive gene that lies downstream of atRA and its receptors in the regulation of neurite outgrowth and cell adhesion in neural as well as non-neural tissues. ARIA is an armadillo (ARM) repeat and BTB domain-containing protein that acts as a positive regulator of ABA response via the modulation of the transcriptional activity of ABF2. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....
gi 288915529   3 RALLYGGMRE-SQPEAEIPlqDTTAEAFTMLLKYIYTGRATLTDEKEEVLLDFLSLAHKYGFPELEDSTsEYLC 75
Cdd:cd18297   39 KSMLSSGSTEaQTPVIEIP--DIRYDIFQLMMQYLYTGGVESLDVAQDDALELLRAASFFQLDGLKRHC-EILL 109

BACK (BTB and C-terminal Kelch) domain found in Kelch-like protein 2 (KLHL2); KLHL2, also called actin-binding protein Mayven, is a novel actin-binding protein predominantly expressed in the brain. It plays a role in the reorganization of the actin cytoskeleton, and promotes growth of cell projections in oligodendrocyte precursors. KLHL2 is a component of a cullin-RING-based BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complex that mediates the ubiquitination of target proteins, such as NPTXR, leading most often to their proteasomal degradation.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529  90 ASLYSLPKLTCMCCMFMDRNAQEVLSSEGFLSLSKTALLNIVLRDSFAAP-EKDIFLALLNWCKHNS---KENHAEIMQA 165
Cdd:cd18512   13 ADMHACTELLNQANTYAEQHFSDVVLSEEFLNLGIEQVCSLIASDKLTISsEEKVFEAVIAWVNHDKevrQEHMAHLMEH 92

                         90       100       110
                 ....*....|....*....|....*....|....*.
gi 288915529 166 VRLPLMSLTELLNVVRPSGLLS-----PDAILDAIK 196
Cdd:cd18512   93 VRLPLLSREYLVQRVEEETLVKnssacKDYLIEAMK 128

BACK (BTB and C-terminal Kelch) domain found in Drosophila melanogaster serine-enriched protein (GPRS) and similar proteins; The family includes uncharacterized Drosophila melanogaster serine-enriched protein (GPRS) and similar proteins.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529  79 NIQNVCMTFDVASLYSLPK----LTCMCCMFMDRNAQEVLSSEGFLSLSKTALLNIVLRDSFAAPEKDIFLALLNWCKHN 154
Cdd:cd18507    1 NVDTVCALLASAERYDQYKctktLVQKVLEFVDKHGTDVLNLPEFLLLPEHVVRLILSRDSLGADEMTKFQAALAWSRKY 80

BACK (BTB and C-terminal Kelch) domain found in BTB/POZ domain-containing protein 2 (BTBD2); BTBD2 is a BTB-domain-containing Kelch-like protein that interacts with DNA topoisomerase 1 (Topo1), a key enzyme in cell survival. BTBD1 and BTBD2 colocalize to cytoplasmic bodies with the RBCC/tripartite motif protein, TRIM5delta.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529  78 LNIQNVCMTFDVASLYSLPKLTCMCCMFMDRNAQEVLSSEGFLSLSKTALLNIVLRDSFAAPEKDIFLALLNWCK----- 152
Cdd:cd18523    1 LRADNAFMLLTQARLFDEPQLASLCLENIDKNTSDAINAEGFTDIDLDTLVAVLERDTLGIREVRLFNAVVRWAEaecqr 80

                         90       100
                 ....*....|....*....|....*.
gi 288915529 153 -------HNSKENHAEIMQAVRLPLM 171
Cdd:cd18523   81 qqlqvtpENKRKVLGKALSLIRFPLM 106

BACK (BTB and C-terminal Kelch) domain found in Kelch-like protein 12 (KLHL12); KLHL12, also termed CUL3-interacting protein 1 (C3IP1), or DKIR, is a substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex that acts as a negative regulator of Wnt signaling pathway and ER-Golgi transport.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529 105 FMDRNAQEVLSSEGFLSLSKTALLNIVLRDSFAAP-EKDIFLALLNWCKHNSKENH---AEIMQAVRLPLMSLTELLNVV 180
Cdd:cd18452   28 FSQKHFPEVVQHEEFMLLSQEEVEKLIKSDEIQVDsEEPVFEAVLNWVKHDKEEREdylPELLQYVRLPLLSPRYITDVV 107

first BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in leucine-zipper-like transcriptional regulator 1 (LZTR-1); LZTR-1 is a golgi BTB-kelch protein that is degraded upon induction of apoptosis. It may also function as a transcriptional regulator that plays a crucial role in embryogenesis. Germline loss-of-function mutations in LZTR-1 predispose to an inherited disorder of multiple schwannomas. LZTR-1 contains two BTB domains. This model corresponds to the first domain. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....
gi 288915529   4 ALLYGGMRESQPEAEIPLQDTTAEAFTMLLKYIYTGRATLTDEKEE------VLL--DFLSLAHKYGFPELEDSTSEYL 74
Cdd:cd18308   77 ADSSSASKNDSPMLEVRLPEADPEAFELILSFIYTDKIDPTRKGQDpgsnevVLLmmDVYRLALQFQMSRLEQLCVQYL 155

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in Kelch repeat and BTB domain-containing protein 8 (KBTBD8); KBTBD8, also called T-cell activation kelch repeat protein (TA-KRP), is a BTB-kelch family protein that is located in the Golgi apparatus and translocates to the spindle apparatus during mitosis. It acts as a substrate-specific adaptor of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex that acts as a regulator of neural crest specification. The BCR(KBTBD8) complex monoubiquitylates NOLC1 and its paralog TCOF1, the mutation of which underlies the neurocristopathy Treacher Collins syndrome. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529   3 RALLYGGMRESQpEAEIPLQDTTAEAFTMLLKYIYTGRATLTDEKEEVLLDFLSLahkYGFPELEDSTSEYLCTILNIQN 82
Cdd:cd18274   50 RSMFTSGLTEST-QKEVRIVGVEAESMHLVLDYAYTSRVTLTEANVQALFTAASI---FQIPSLQDQCAQFMISRLDPQN 125

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in Drosophila melanogaster protein roadkill and similar proteins; Drosophila melanogaster protein roadkill, also called Hh-induced MATH and BTB domain-containing protein (HIB), is a hedgehog-induced BTB protein that modulates hedgehog signaling by degrading Ci/Gli transcription factor. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529   3 RALLYGGMRESQPEAE----IPLQDTTAEAFTMLLKYIYTGRATLTDEKEEvllDFLSLAHKYGFPELEDSTSEYLCTIL 78
Cdd:cd18345   40 RSPVFNAMFEHEMEERkqnrVEITDVDHEVMREMLRFIYTGKAPNLDKMAD---DLLAAADKYALERLKVMCEEALCSNL 116

                 ....*
gi 288915529  79 NIQNV 83
Cdd:cd18345  117 SVENV 121

second BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in Ankyrin repeat and BTB/POZ domain-containing protein 1 (ABTB1); ABTB1, also called elongation factor 1A-binding protein or bood POZ containing gene type 1 (BPOZ-1), is an anti-proliferative factor that may act as a mediator of the phosphatase and tensin homolog (PTEN) growth-suppressive signaling pathway. It may play a role in developmental processes. ABTB1 contains an ankyrin repeat and two BTB domains. This model corresponds to the second BTB domain. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529   3 RALLYGGMRESQPE-AEIP---LQDTTAEAFTMLLKYIYTGRATLTDekeEVLLDFLSLAHKYGFPELEDstseyLCTIL 78
Cdd:cd18296   44 KALLRDHFAESEENnGSIPvvtLHDVSPEVFAIVLYYIYTDDTDLPP---ENAYDVLYVADMYLLPGLKR-----LCGTV 115

                 ....*.
gi 288915529  79 NIQNVC 84
Cdd:cd18296  116 LAQSLD 121

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in rabankyrin-5 (Rank-5); Rank-5, also called ankyrin repeat and FYVE domain-containing protein 1 (ANKFY1) or ankyrin repeats hooked to a zinc finger motif (ANKHZN), is a Rab5 effector that regulates and coordinates different endocytic mechanisms. It contains an N-terminal BTB domain, followed by a BACK domain, several ankyrin (ANK) repeats and a C-terminal FYVE domain. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*
gi 288915529  18 EIPLQDTTAEAFTMLLKYIYTGRATLTDEkEEVLLDFLSLAHKYGFPELEDSTSEYLCTILNIQN 82
Cdd:cd18303   54 ELDLSDISYEVVLALLRWLYTDELDLTLD-DEFLLELMKAAKRFQLTDLVERCERALMSSVNVDN 117

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in speckle-type POZ protein-like (SPOPL); SPOPL, also called HIB homolog 2 or Roadkill homolog 2, is a component of a cullin-RING-based BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complexes that mediate the ubiquitination and subsequent proteasomal degradation of target proteins. The complexes may contain homodimeric SPOPL or the heterodimers formed by speckle-type POZ protein (SPOP) and SPOPL, which are less efficient than ubiquitin ligase complexes containing only SPOP. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|...
gi 288915529  10 MRESQpEAEIPLQDTTAEAFTMLLKYIYTGRATLTDEKEEVLldfLSLAHKYGFPELEDSTSEYLCTILNIQN 82
Cdd:cd18343   55 MEESK-KNRVEINDVDPEVFKEMMRFIYTGKAPNLDKMADNL---LAAADKYALERLKVMCEEALCNNLSVEN 123

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in Kelch-like protein 7 (KLHL7); KLHL7 is a component of a Cul3-based E3 ubiquitin ligase complex and is involved in the ubiquitination of target proteins for proteasome-mediated degradation. Mutations in KLHL7 causes autosomal-dominant retinitis pigmentosa. It contains a BTB domain and kelch repeats, characteristics of a kelch family protein. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529   3 RALLYGGMRESQpEAEIPLQDTTAEAFTMLLKYIYTGRATLTDEKEEVLLDflsLAHKYGFPELEDSTSEYLCTILNIQN 82
Cdd:cd18237   48 HLMFTSNMTESK-SSEVELKDAEPDIIELLVEFAYTARISVNSNNVQSLLD---AANQYQIEPVKKMCVDFLKEQLDASN 123

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in BTB/POZ domain-containing protein 1 (BTBD1); BTBD1, also called Hepatitis C virus NS5A-transactivated protein 8 or HCV NS5A-transactivated protein 8, is a BTB-domain-containing Kelch-like protein that is expressed in skeletal muscle and interacts with DNA topoisomerase 1 (Topo1), a key enzyme of cell survival. BTBD1 and BTBD2 colocalize to cytoplasmic bodies with the RBCC/tripartite motif protein, TRIM5delta. BTBD1 may serve as substrate-specific adaptor of an E3 ubiquitin-protein ligase complex that mediates the ubiquitination and subsequent proteasomal degradation of target proteins. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....
gi 288915529   4 ALLYGGMRESQPEAEIPlqDTTAEAFTMLLKYIYTGRATLtdeKEEVLLDFLSLAHKYGFPELEDSTSEYLCTILNIQN 82
Cdd:cd18346   60 AMFNGGMATTSAEIELP--DVEPAAFLALLRFLYSDEVQI---GPETVMTTLYTAKKYAVPALEAHCVEFLTKHLRADN 133

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in Kelch-like protein 8 (KLHL8); KLHL8 is a substrate-specific adaptor of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex required for the ubiquitination and degradation of rapsyn, a postsynaptic protein required for clustering of nicotinic acetylcholine receptors (nAChRs) at the neuromuscular junction. It contains a BTB domain and kelch repeats, characteristics of a kelch family protein. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529   3 RALLYGGMRESQpEAEIPLQDTTAEAFTMLLKYIYTGRATLTDEKEEVLLDFLSLahkYGFPELEDSTSEYLCTILNIQN 82
Cdd:cd18238   42 RAMFTSEMAESK-QDSITIKDIDEEAVELLVDFAYTGKLTLTVDNVQSLLYAASL---LQVEEVAKACCEFMKDHLHPSN 117

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in BTB/POZ domain-containing protein 6 (BTBD6); BTBD6, also termed lens BTB domain protein, is a BTB-domain-containing Kelch-like protein required for proper embryogenesis and plays an essential evolutionary conserved role during neuronal development. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*
gi 288915529   4 ALLYGGMRESQPEAEIPlqDTTAEAFTMLLKYIYTGRATLtdeKEEVLLDFLSLAHKYGFPELEDSTSEYLCTIL 78
Cdd:cd18349   40 AMFYGDLAEVKSEIHIP--DVEPAAFLILLKYMYSDEIDL---EADTVLATLYAAKKYIVPALAKACVNFLETSL 109

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in speckle-type POZ protein (SPOP) and similar proteins; This family includes speckle-type POZ protein (SPOP), speckle-type POZ protein-like (SPOPL), TD and POZ domain-containing proteins (TDPOZ), Drosophila melanogaster protein roadkill and similar proteins. Both, SPOP and SPOPL, serve as adaptors of cullin-RING-based BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complexes that mediate the ubiquitination and proteasomal degradation of target proteins. TDPOZ is a family of bipartite animal and plant proteins that contain a tumor necrosis factor receptor-associated factor (TRAF) domain (TD) and a POZ/BTB domains. TDPOZ proteins may be nuclear scaffold proteins probably involved in transcription regulation in early development and other cellular processes. Drosophila melanogaster protein roadkill, also called Hh-induced MATH and BTB domain-containing protein (HIB), is a hedgehog-induced BTB protein that modulates hedgehog signaling by degrading Ci/Gli transcription factor. The BTB/POZ domain is a common protein-protein interaction motif of about 100 amino acids.

                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
gi 288915529   3 RALLYGGMRESQpEAEIPLQDTTAEAFTMLLKYIYTGRATLTDEKEEvllDFLSLAHKYGFPELEDSTSEYLCTILNIQN 82
Cdd:cd18279   45 SAMFEHEMEESK-KNRVEINDVDPEVFKEMMRFIYTGKAPNLDKMAD---DLLAAADKYALERLKVMCEDALCSNLSVEN 120

BACK (BTB and C-terminal Kelch) domain found in Kelch-like protein 1 (KLHL1); KLHL1 is a neuronal actin-binding protein that modulates voltage-gated CaV2.1 (P/Q-type) and CaV3.2 (alpha1H T-type) calcium channels. It may play a role in organizing the actin cytoskeleton in brain cells. KLHL1 contains a BTB domain and kelch repeat domains, characteristics of a kelch family protein.

                         10        20        30        40        50        60
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*..
gi 288915529 109 NAQEVLSSEGFLSLSKTALLNIVLRDSFAAPEKD-IFLALLNWCKHNSKE---NHAEIMQAVRLPLM 171
Cdd:cd18509   33 NIMEVIRNQEFLLLPAEELHKLLASDDVNVPDEEtIFHALMMWVKYDMQRrcsDLSMLLAYIRLPLL 99

BACK (BTB and C-terminal Kelch) domain found in Kelch-like ECH-associated protein 1 (KEAP1); KEAP1, also termed cytosolic inhibitor of Nrf2 (INrf2), or Kelch-like protein 19 (KLHL19), is a redox-regulated substrate adaptor protein for a Cullin3-dependent ubiquitin ligase complex that targets NFE2L2/NRF2 for ubiquitination and degradation by the proteasome, thus resulting in the suppression of its transcriptional activity and the repression of antioxidant response element-mediated detoxifying enzyme gene expression.

                         10        20        30        40        50
                 ....*....|....*....|....*....|....*....|....*....|....*....
gi 288915529 105 FMDRNAQEVLSSEGFLSLSKTALLNIVLRDSFAAP-EKDIFLALLNWCKHNSKENHAEI 162
Cdd:cd18458   29 YIYMHFSEVSQSEEFFNLSPCQLVALISRDELNVRcESEVYNAVIRWVKYDEENRRQYL 87