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别名
- DJ4
- DnaJ
- DnaJ (Hsp40) homolog subfamily B member 6
- DNAJ B6
- DnaJ homolog subfamily B member 6
- DnaJ like 2 protein
- DNAJB6
- DNJB6_HUMAN
- Heat shock protein J2
- HHDJ1
- HSJ-2
- HSJ2
- LGMD1E
- MRJ
- MSJ-1
- MSJ1
see all
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功能
Plays an indispensable role in the organization of KRT8/KRT18 filaments. Acts as an endogenous molecular chaperone for neuronal proteins including huntingtin. Suppresses aggregation and toxicity of polyglutamine-containing, aggregation-prone proteins. Isoform B but not isoform A inhibits huntingtin aggregation. Has a stimulatory effect on the ATPase activity of HSP70 in a dose-dependent and time-dependent manner and hence acts as a co-chaperone of HSP70. Also reduces cellular toxicity and caspase-3 activity.
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组织特异性
Widely expressed. Highest levels in testis and brain, and lower levels in heart, spleen, intestine, ovary, placenta, lung, kidney, pancreas, thymus, prostate, skeletal muscle, liver and leukocytes. In testis, expressed in germ cells in the earlier stages of differentiation pathway as well as in spermatids. In brain, expressed at a higher level in hippocampus and thalamus and a lower level in amygdala, substantia nigra, corpus callosum and caudate nucleus.
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疾病相关
Limb-girdle muscular dystrophy 1E
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序列相似性
Contains 1 J domain.
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结构域
The antiaggregation activity of isoform B resides in the serine-rich region and the C-terminus.
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细胞定位
Cytoplasm, perinuclear region. Nucleus. Cytoplasm, myofibril, sarcomere, Z line.
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Information by UniProt