heat shock protein family B (small) member 8
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The HSPB8 gene provides instructions for making a protein called heat shock protein beta-8. This protein is a member of the heat shock protein family, which helps protect cells that are under stress from factors such as infection, inflammation, exposure to toxins, elevated temperature, injury, and disease. Heat shock proteins block signals that lead cells to self-destruct (undergo apoptosis). In addition, they appear to be involved in cell movement, stabilizing the cell's structural framework (the cytoskeleton), folding and stabilizing newly produced proteins, and repairing damaged proteins. Heat shock proteins also seem to play a role in the tensing of muscle fibers (muscle contraction).
Normal function
The HSPB8 gene provides instructions for making a protein called heat shock protein beta-8. This protein is a member of the heat shock protein family, which helps protect cells that are under stress from factors such as infection, inflammation, exposure to toxins, elevated temperature, injury, and disease. Heat shock proteins block signals that lead cells to self-destruct (undergo apoptosis). In addition, they appear to be involved in cell movement, stabilizing the cell's structural framework (the cytoskeleton), folding and stabilizing newly produced proteins, and repairing damaged proteins. Heat shock proteins also seem to play a role in the tensing of muscle fibers (muscle contraction).
Heat shock protein beta-8 is found in cells throughout the body, where it helps fold newly produced proteins and refold damaged proteins. Heat shock protein beta-8 seems to interact with a related protein called heat shock protein beta-1, which is produced from the HSPB1 gene. In nerve cells (neurons), heat shock protein beta-1 helps to organize a network of molecular threads called neurofilaments that maintain the structure of a part of the neuron called the axon. This allows neurons to transmit nerve impulses efficiently. The specific role that heat shock protein beta-8 plays in axons is unclear.
Conditions referring to this gene or chromosome
Other names
H11
heat shock protein beta-8
HSP22
HspB8
HSPB8_HUMAN
protein kinase H11
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Weiterführende Lektüre und Referenzen
- heat shock protein family B (small) member 8; MedlinePlus, National Library of Medicine.
- Bird TD. Charcot-Marie-Tooth Hereditary Neuropathy Overview. 1998 Sep 28 [updated 2025 Nov 20]. In: Adam MP, Bick S, Mirzaa GM, Wallace SE, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2026. Available from http://www.ncbi.nlm.nih.gov/books/NBK1358/
- Datskevich PN, Nefedova VV, Sudnitsyna MV, Gusev NB. Mutations of small heat shock proteins and human congenital diseases. Biochemistry (Mosc). 2012 Dec;77(13):1500-14. doi: 10.1134/S0006297912130081.
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- Drew AP, Blair IP, Nicholson GA. Molecular genetics and mechanisms of disease in distal hereditary motor neuropathies: insights directing future genetic studies. Curr Mol Med. 2011 Nov;11(8):650-65. doi: 10.2174/156652411797536714.
- Fontaine JM, Sun X, Hoppe AD, Simon S, Vicart P, Welsh MJ, Benndorf R. Abnormal small heat shock protein interactions involving neuropathy-associated HSP22 (HSPB8) mutants. FASEB J. 2006 Oct;20(12):2168-70. doi: 10.1096/fj.06-5911fje. Epub 2006 Aug 25.
- Hu Z, Chen L, Zhang J, Li T, Tang J, Xu N, Wang X. Structure, function, property, and role in neurologic diseases and other diseases of the sHsp22. J Neurosci Res. 2007 Aug 1;85(10):2071-9. doi: 10.1002/jnr.21231.
- Irobi J, Van Impe K, Seeman P, Jordanova A, Dierick I, Verpoorten N, Michalik A, De Vriendt E, Jacobs A, Van Gerwen V, Vennekens K, Mazanec R, Tournev I, Hilton-Jones D, Talbot K, Kremensky I, Van Den Bosch L, Robberecht W, Van Vandekerckhove J, Van Broeckhoven C, Gettemans J, De Jonghe P, Timmerman V. Hot-spot residue in small heat-shock protein 22 causes distal motor neuropathy. Nat Genet. 2004 Jun;36(6):597-601. doi: 10.1038/ng1328. Epub 2004 May 2.
- Nefedova VV, Muranova LK, Sudnitsyna MV, Ryzhavskaya AS, Gusev NB. Small Heat Shock Proteins and Distal Hereditary Neuropathies. Biochemistry (Mosc). 2015 Dec;80(13):1734-47. doi: 10.1134/S000629791513009X.
- Shemetov AA, Seit-Nebi AS, Gusev NB. Structure, properties, and functions of the human small heat-shock protein HSP22 (HspB8, H11, E2IG1): a critical review. J Neurosci Res. 2008 Feb 1;86(2):264-9. doi: 10.1002/jnr.21441.
- Tang BS, Zhao GH, Luo W, Xia K, Cai F, Pan Q, Zhang RX, Zhang FF, Liu XM, Chen B, Zhang C, Shen L, Jiang H, Long ZG, Dai HP. Small heat-shock protein 22 mutated in autosomal dominant Charcot-Marie-Tooth disease type 2L. Hum Genet. 2005 Feb;116(3):222-4. doi: 10.1007/s00439-004-1218-3. Epub 2004 Nov 23.
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30. Sept. 2025 | Neueste Version
1 Mar 2025 | Ursprünglich veröffentlicht
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