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Sarcoglycanopathy

MedGen UID:
424705
Concept ID:
C2936331
Disease or Syndrome
Synonym: Sarcoglycanopathies
 
Related genes: SGCG, SGCA
 
Monarch Initiative: MONDO:0016140
Orphanet: ORPHA207052

Definition

Deficiencies or mutations in the genes for the sarcoglycan complex subunits. A variety of phenotypes are associated with these mutations including a subgroup of autosomal recessive limb girdle muscular dystrophies, cardiomyopathies, and respiratory deficiency. [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Alhammad RM, Alrehaili ML, Albulaihe HM, Aljereish SS, Alanazy MH
BMC Neurol 2024 Sep 4;24(1):312. doi: 10.1186/s12883-024-03838-2. PMID: 39232665Free PMC Article
Carson L, Merrick D
Ir J Med Sci 2022 Dec;191(6):2743-2750. Epub 2022 Jan 18 doi: 10.1007/s11845-021-02855-1. PMID: 35040091
Cotta A, Carvalho E, da-Cunha-Júnior AL, Paim JF, Navarro MM, Valicek J, Menezes MM, Nunes SV, Xavier Neto R, Takata RI, Vargas AP
Arq Neuropsiquiatr 2014 Sep;72(9):721-34. doi: 10.1590/0004-282x20140110. PMID: 25252238

Recent clinical studies

Etiology

Angelini C
Acta Myol 2020 Dec;39(4):207-217. Epub 2020 Dec 1 doi: 10.36185/2532-1900-024. PMID: 33458576Free PMC Article
Ten Dam L, Frankhuizen WS, Linssen WHJP, Straathof CS, Niks EH, Faber K, Fock A, Kuks JB, Brusse E, de Coo R, Voermans N, Verrips A, Hoogendijk JE, van der Pol L, Westra D, de Visser M, van der Kooi AJ, Ginjaar I
Clin Genet 2019 Aug;96(2):126-133. Epub 2019 May 6 doi: 10.1111/cge.13544. PMID: 30919934
Angelini C, Giaretta L, Marozzo R
Expert Rev Neurother 2018 Sep;18(9):693-703. Epub 2018 Aug 21 doi: 10.1080/14737175.2018.1508997. PMID: 30084281
Fayssoil A, Nardi O, Annane D, Orlikowski D
Acta Neurol Belg 2014 Dec;114(4):257-9. Epub 2014 Jan 25 doi: 10.1007/s13760-013-0276-5. PMID: 24464767
Gulati S, Leekha S, Sharma MC, Kalra V
Indian Pediatr 2003 Nov;40(11):1077-81. PMID: 14660840

Diagnosis

Alonso-Pérez J, González-Quereda L, Bello L, Guglieri M, Straub V, Gallano P, Semplicini C, Pegoraro E, Zangaro V, Nascimento A, Ortez C, Comi GP, Dam LT, De Visser M, van der Kooi AJ, Garrido C, Santos M, Schara U, Gangfuß A, Løkken N, Storgaard JH, Vissing J, Schoser B, Dekomien G, Udd B, Palmio J, D'Amico A, Politano L, Nigro V, Bruno C, Panicucci C, Sarkozy A, Abdel-Mannan O, Alonso-Jimenez A, Claeys KG, Gomez-Andrés D, Munell F, Costa-Comellas L, Haberlová J, Rohlenová M, Elke V, De Bleecker JL, Dominguez-González C, Tasca G, Weiss C, Deconinck N, Fernández-Torrón R, López de Munain A, Camacho-Salas A, Melegh B, Hadzsiev K, Leonardis L, Koritnik B, Garibaldi M, de Leon-Hernández JC, Malfatti E, Fraga-Bau A, Richard I, Illa I, Díaz-Manera J
Brain 2020 Sep 1;143(9):2696-2708. doi: 10.1093/brain/awaa228. PMID: 32875335
Ten Dam L, Frankhuizen WS, Linssen WHJP, Straathof CS, Niks EH, Faber K, Fock A, Kuks JB, Brusse E, de Coo R, Voermans N, Verrips A, Hoogendijk JE, van der Pol L, Westra D, de Visser M, van der Kooi AJ, Ginjaar I
Clin Genet 2019 Aug;96(2):126-133. Epub 2019 May 6 doi: 10.1111/cge.13544. PMID: 30919934
Angelini C, Giaretta L, Marozzo R
Expert Rev Neurother 2018 Sep;18(9):693-703. Epub 2018 Aug 21 doi: 10.1080/14737175.2018.1508997. PMID: 30084281
Kapoor S, Tatke M, Aggarwal S, Gupta A
Indian J Pediatr 2005 Jan;72(1):71-4. doi: 10.1007/BF02760585. PMID: 15684453
Gulati S, Leekha S, Sharma MC, Kalra V
Indian Pediatr 2003 Nov;40(11):1077-81. PMID: 14660840

Therapy

Górecki DC
Curr Opin Pharmacol 2019 Aug;47:40-45. Epub 2019 Mar 20 doi: 10.1016/j.coph.2019.02.003. PMID: 30901735
Maruyama R, Yokota T
Methods Mol Biol 2018;1828:79-90. doi: 10.1007/978-1-4939-8651-4_5. PMID: 30171536
Shimo T, Maruyama R, Yokota T
Methods Mol Biol 2018;1687:143-155. doi: 10.1007/978-1-4939-7374-3_10. PMID: 29067661
Herson S, Hentati F, Rigolet A, Behin A, Romero NB, Leturcq F, Laforêt P, Maisonobe T, Amouri R, Haddad H, Audit M, Montus M, Masurier C, Gjata B, Georger C, Cheraï M, Carlier P, Hogrel JY, Herson A, Allenbach Y, Lemoine FM, Klatzmann D, Sweeney HL, Mulligan RC, Eymard B, Caizergues D, Voït T, Benveniste O
Brain 2012 Feb;135(Pt 2):483-92. Epub 2012 Jan 11 doi: 10.1093/brain/awr342. PMID: 22240777
Baumeister SK, Todorovic S, Milić-Rasić V, Dekomien G, Lochmüller H, Walter MC
Neuromuscul Disord 2009 Feb;19(2):167-71. Epub 2009 Jan 23 doi: 10.1016/j.nmd.2008.11.010. PMID: 19167890

Prognosis

Xie Z, Liu C, Yu H, Xie Z, Sun C, Zhu Y, Hu X, Bai L, Wei L, Sun P, Lu Y, Lu Y, Zhao Y, Zhang W, Wang Z, Meng L, Yuan Y
Orphanet J Rare Dis 2024 Mar 14;19(1):123. doi: 10.1186/s13023-024-03128-7. PMID: 38486238Free PMC Article
Fayssoil A, Nardi O, Annane D, Orlikowski D
Acta Neurol Belg 2014 Dec;114(4):257-9. Epub 2014 Jan 25 doi: 10.1007/s13760-013-0276-5. PMID: 24464767
Gulati S, Leekha S, Sharma MC, Kalra V
Indian Pediatr 2003 Nov;40(11):1077-81. PMID: 14660840
Gordon ES, Hoffman EP
Curr Opin Neurol 2001 Oct;14(5):567-73. doi: 10.1097/00019052-200110000-00004. PMID: 11562567
Ozawa E, Noguchi S, Mizuno Y, Hagiwara Y, Yoshida M
Muscle Nerve 1998 Apr;21(4):421-38. doi: 10.1002/(sici)1097-4598(199804)21:4<421::aid-mus1>3.0.co;2-b. PMID: 9533777

Clinical prediction guides

Xie Z, Liu C, Yu H, Xie Z, Sun C, Zhu Y, Hu X, Bai L, Wei L, Sun P, Lu Y, Lu Y, Zhao Y, Zhang W, Wang Z, Meng L, Yuan Y
Orphanet J Rare Dis 2024 Mar 14;19(1):123. doi: 10.1186/s13023-024-03128-7. PMID: 38486238Free PMC Article
Alonso-Pérez J, González-Quereda L, Bello L, Guglieri M, Straub V, Gallano P, Semplicini C, Pegoraro E, Zangaro V, Nascimento A, Ortez C, Comi GP, Dam LT, De Visser M, van der Kooi AJ, Garrido C, Santos M, Schara U, Gangfuß A, Løkken N, Storgaard JH, Vissing J, Schoser B, Dekomien G, Udd B, Palmio J, D'Amico A, Politano L, Nigro V, Bruno C, Panicucci C, Sarkozy A, Abdel-Mannan O, Alonso-Jimenez A, Claeys KG, Gomez-Andrés D, Munell F, Costa-Comellas L, Haberlová J, Rohlenová M, Elke V, De Bleecker JL, Dominguez-González C, Tasca G, Weiss C, Deconinck N, Fernández-Torrón R, López de Munain A, Camacho-Salas A, Melegh B, Hadzsiev K, Leonardis L, Koritnik B, Garibaldi M, de Leon-Hernández JC, Malfatti E, Fraga-Bau A, Richard I, Illa I, Díaz-Manera J
Brain 2020 Sep 1;143(9):2696-2708. doi: 10.1093/brain/awaa228. PMID: 32875335
Angelini C, Tasca E
Neuromuscul Disord 2012 Dec;22 Suppl 3(3-3):S214-20. doi: 10.1016/j.nmd.2012.10.010. PMID: 23182642Free PMC Article
Nalini A, Gayathri N, Thaha F, Das S, Shylashree S
Neurol India 2010 Sep-Oct;58(5):691-6. doi: 10.4103/0028-3886.72164. PMID: 21045489
Ozawa E, Noguchi S, Mizuno Y, Hagiwara Y, Yoshida M
Muscle Nerve 1998 Apr;21(4):421-38. doi: 10.1002/(sici)1097-4598(199804)21:4<421::aid-mus1>3.0.co;2-b. PMID: 9533777

Recent systematic reviews

Carson L, Merrick D
Ir J Med Sci 2022 Dec;191(6):2743-2750. Epub 2022 Jan 18 doi: 10.1007/s11845-021-02855-1. PMID: 35040091

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