DNAJB2 P25686

DnaJ同源亚家族B成员2 (热休克40 kDa蛋白3) (热休克蛋白J1) (HSJ-1)

功能描述

作为共伴侣蛋白发挥作用，调节HSP70/heat shock protein 70家族chaperones的底物结合并激活其ATPase活性 (PubMed:22219199, PubMed:7957263)。同时，也促进错误折叠蛋白的泛素依赖性蛋白酶体降解 (PubMed:15936278, PubMed:21625540)。因此，可能调节HTT、MC4R、PRKN、RHO和SOD1等错误折叠蛋白的聚集并促进其功能恢复，对许多生物过程至关重要 (PubMed:12754272, PubMed:20889486, PubMed:21719532, PubMed:22396390, PubMed:24023695)。定位于内质网膜的Isoform 1可能特异性地在错误折叠蛋白的内质网相关蛋白降解中发挥作用 (PubMed:15936278)。 {ECO:0000269|PubMed:12754272, ECO:0000269|PubMed:15936278, ECO:0000269|PubMed:20889486, ECO:0000269|PubMed:21625540, ECO:0000269|PubMed:21719532, ECO:0000269|PubMed:22219199, ECO:0000269|PubMed:22396390, ECO:0000269|PubMed:24023695, ECO:0000269|PubMed:7957263}。

组织特异性

More abundantly expressed in neocortex, cerebellum, spinal cord and retina where it is expressed by neuronal cells (at protein level) (PubMed:12754272, PubMed:1599432). Detected at much lower level in non-neuronal tissues including kidney, lung, heart, skeletal muscle, spleen and testis (at protein level) (PubMed:12754272, PubMed:1599432). Isoform 1 is more abundant in neocortex and cerebellum compared to isoform 2 (at protein level) (PubMed:12754272).

亚细胞定位

[Isoform 2]: Cytoplasm