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Protein Aggregation, Pathological

MedGen UID:
827241
Concept ID:
C3850147
Molecular Function
Synonyms: Aggregation, Pathological Protein; Pathological Protein Aggregation

Definition

A biochemical phenomenon in which misfolded proteins aggregate either intra- or extracellularly. Triggered by factors such as MUTATION; POST-TRANSLATIONAL MODIFICATIONS, and environmental stress, it is generally associated with ALZHEIMER DISEASE; PARKINSON DISEASE; HUNTINGTON DISEASE; and TYPE 2 DIABETES MELLITUS. [from MeSH]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProtein Aggregation, Pathological

Professional guidelines

PubMed

Fan Y, Chen Z, Zhang M
J Transl Med 2022 Jun 27;20(1):291. doi: 10.1186/s12967-022-03493-6. PMID: 35761337Free PMC Article
Pichla M, Bartosz G, Sadowska-Bartosz I
Oxid Med Cell Longev 2020;2020:3534570. Epub 2020 Oct 13 doi: 10.1155/2020/3534570. PMID: 33123310Free PMC Article

Recent clinical studies

Etiology

Ashrafian H, Zadeh EH, Khan RH
Int J Biol Macromol 2021 Jan 15;167:382-394. Epub 2020 Dec 2 doi: 10.1016/j.ijbiomac.2020.11.192. PMID: 33278431
Picken MM
Acta Haematol 2020;143(4):322-334. Epub 2020 May 11 doi: 10.1159/000506696. PMID: 32392555
Whitechurch BC, Welton JM, Collins SJ, Lawson VA
Adv Neurobiol 2017;15:335-364. doi: 10.1007/978-3-319-57193-5_13. PMID: 28674988
Balchin D, Hayer-Hartl M, Hartl FU
Science 2016 Jul 1;353(6294):aac4354. doi: 10.1126/science.aac4354. PMID: 27365453
Molliex A, Temirov J, Lee J, Coughlin M, Kanagaraj AP, Kim HJ, Mittag T, Taylor JP
Cell 2015 Sep 24;163(1):123-33. doi: 10.1016/j.cell.2015.09.015. PMID: 26406374Free PMC Article

Diagnosis

Wisniowski B, Wechalekar A
Acta Haematol 2020;143(4):312-321. Epub 2020 Jun 16 doi: 10.1159/000508022. PMID: 32544917
Picken MM
Acta Haematol 2020;143(4):322-334. Epub 2020 May 11 doi: 10.1159/000506696. PMID: 32392555
Vaquer-Alicea J, Diamond MI
Annu Rev Biochem 2019 Jun 20;88:785-810. Epub 2019 Mar 27 doi: 10.1146/annurev-biochem-061516-045049. PMID: 30917002
Soto C, Pritzkow S
Nat Neurosci 2018 Oct;21(10):1332-1340. Epub 2018 Sep 24 doi: 10.1038/s41593-018-0235-9. PMID: 30250260Free PMC Article
Cooper JF, Van Raamsdonk JM
J Parkinsons Dis 2018;8(1):17-32. doi: 10.3233/JPD-171258. PMID: 29480229Free PMC Article

Therapy

Zhao Y, Zhang Z, Qin S, Fan W, Li W, Liu J, Wang S, Xu Z, Zhao M
Neural Plast 2021;2021:9926445. Epub 2021 Jun 15 doi: 10.1155/2021/9926445. PMID: 34221005Free PMC Article
Dias AP, da Silva Santos S, da Silva JV, Parise-Filho R, Igne Ferreira E, Seoud OE, Giarolla J
Int J Pharm 2020 Jan 5;573:118814. Epub 2019 Nov 20 doi: 10.1016/j.ijpharm.2019.118814. PMID: 31759101
Venegas C, Kumar S, Franklin BS, Dierkes T, Brinkschulte R, Tejera D, Vieira-Saecker A, Schwartz S, Santarelli F, Kummer MP, Griep A, Gelpi E, Beilharz M, Riedel D, Golenbock DT, Geyer M, Walter J, Latz E, Heneka MT
Nature 2017 Dec 20;552(7685):355-361. doi: 10.1038/nature25158. PMID: 29293211
Sevigny J, Chiao P, Bussière T, Weinreb PH, Williams L, Maier M, Dunstan R, Salloway S, Chen T, Ling Y, O'Gorman J, Qian F, Arastu M, Li M, Chollate S, Brennan MS, Quintero-Monzon O, Scannevin RH, Arnold HM, Engber T, Rhodes K, Ferrero J, Hang Y, Mikulskis A, Grimm J, Hock C, Nitsch RM, Sandrock A
Nature 2016 Sep 1;537(7618):50-6. doi: 10.1038/nature19323. PMID: 27582220
Zhao L, Chen XJ, Zhu J, Xi YB, Yang X, Hu LD, Ouyang H, Patel SH, Jin X, Lin D, Wu F, Flagg K, Cai H, Li G, Cao G, Lin Y, Chen D, Wen C, Chung C, Wang Y, Qiu A, Yeh E, Wang W, Hu X, Grob S, Abagyan R, Su Z, Tjondro HC, Zhao XJ, Luo H, Hou R, Jefferson J, Perry P, Gao W, Kozak I, Granet D, Li Y, Sun X, Wang J, Zhang L, Liu Y, Yan YB, Zhang K
Nature 2015 Jul 30;523(7562):607-11. Epub 2015 Jul 22 doi: 10.1038/nature14650. PMID: 26200341

Prognosis

Sengupta U, Kayed R
Prog Neurobiol 2022 Jul;214:102270. Epub 2022 Apr 18 doi: 10.1016/j.pneurobio.2022.102270. PMID: 35447272
Pinheiro F, Santos J, Ventura S
J Mol Biol 2021 Oct 1;433(20):167059. Epub 2021 May 21 doi: 10.1016/j.jmb.2021.167059. PMID: 34023402
Dujardin S, Commins C, Lathuiliere A, Beerepoot P, Fernandes AR, Kamath TV, De Los Santos MB, Klickstein N, Corjuc DL, Corjuc BT, Dooley PM, Viode A, Oakley DH, Moore BD, Mullin K, Jean-Gilles D, Clark R, Atchison K, Moore R, Chibnik LB, Tanzi RE, Frosch MP, Serrano-Pozo A, Elwood F, Steen JA, Kennedy ME, Hyman BT
Nat Med 2020 Aug;26(8):1256-1263. Epub 2020 Jun 22 doi: 10.1038/s41591-020-0938-9. PMID: 32572268Free PMC Article
Wisniowski B, Wechalekar A
Acta Haematol 2020;143(4):312-321. Epub 2020 Jun 16 doi: 10.1159/000508022. PMID: 32544917
Holmes BB, Furman JL, Mahan TE, Yamasaki TR, Mirbaha H, Eades WC, Belaygorod L, Cairns NJ, Holtzman DM, Diamond MI
Proc Natl Acad Sci U S A 2014 Oct 14;111(41):E4376-85. Epub 2014 Sep 26 doi: 10.1073/pnas.1411649111. PMID: 25261551Free PMC Article

Clinical prediction guides

Sengupta U, Kayed R
Prog Neurobiol 2022 Jul;214:102270. Epub 2022 Apr 18 doi: 10.1016/j.pneurobio.2022.102270. PMID: 35447272
Wisniowski B, Wechalekar A
Acta Haematol 2020;143(4):312-321. Epub 2020 Jun 16 doi: 10.1159/000508022. PMID: 32544917
Wegmann S, Eftekharzadeh B, Tepper K, Zoltowska KM, Bennett RE, Dujardin S, Laskowski PR, MacKenzie D, Kamath T, Commins C, Vanderburg C, Roe AD, Fan Z, Molliex AM, Hernandez-Vega A, Muller D, Hyman AA, Mandelkow E, Taylor JP, Hyman BT
EMBO J 2018 Apr 3;37(7) Epub 2018 Feb 22 doi: 10.15252/embj.201798049. PMID: 29472250Free PMC Article
Sevigny J, Chiao P, Bussière T, Weinreb PH, Williams L, Maier M, Dunstan R, Salloway S, Chen T, Ling Y, O'Gorman J, Qian F, Arastu M, Li M, Chollate S, Brennan MS, Quintero-Monzon O, Scannevin RH, Arnold HM, Engber T, Rhodes K, Ferrero J, Hang Y, Mikulskis A, Grimm J, Hock C, Nitsch RM, Sandrock A
Nature 2016 Sep 1;537(7618):50-6. doi: 10.1038/nature19323. PMID: 27582220
Holmes BB, Furman JL, Mahan TE, Yamasaki TR, Mirbaha H, Eades WC, Belaygorod L, Cairns NJ, Holtzman DM, Diamond MI
Proc Natl Acad Sci U S A 2014 Oct 14;111(41):E4376-85. Epub 2014 Sep 26 doi: 10.1073/pnas.1411649111. PMID: 25261551Free PMC Article

Recent systematic reviews

Muiño E, Fernández-Cadenas I, Arboix A
Int J Mol Sci 2021 Jul 8;22(14) doi: 10.3390/ijms22147357. PMID: 34298974Free PMC Article
Farace C, Fenu G, Lintas S, Oggiano R, Pisano A, Sabalic A, Solinas G, Bocca B, Forte G, Madeddu R
Neurotoxicology 2020 Dec;81:80-88. Epub 2020 Sep 14 doi: 10.1016/j.neuro.2020.09.003. PMID: 32941938
Mroczko B, Groblewska M, Litman-Zawadzka A
Int J Mol Sci 2019 Sep 20;20(19) doi: 10.3390/ijms20194661. PMID: 31547024Free PMC Article

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