| 别名 | 60 kDa heat shock protein mitochondrial |
| 基因名称 | Endogenous |
| 蛋白名称 | HSPD1 |
| 推荐应用 | |
| 反应物种 | Hsp60 |
| 存储缓冲液 | IHC 1:1000-1:4000;WB 1:1000-1:5000;IF 1:200-1:1000;ELISA 1:5000-1:20000;IP 1:50-1:200, |
| Human Gene Link | HSPD1;HSP60;60 kDa heat shock protein;mitochondrial;60 kDa chaperonin;Chaperonin 60;CPN60;Heat shock protein 60;HSP-60;Hsp60;HuCHA60;Mitochondrial matrix protein P1;P60 lymphocyte protein |
| Human Swissprot No. | 3329 |
| Mouse Gene ID | P10809 |
| Mouse Swissprot No. | 15510 |
| Rat Gene ID | 63868 |
| Rat Swissprot No. | P63039 |
| 特异性 | Monoclonal |
| 稀释度 | Unmodified |
| 参考分子量 | 60kD |
| 预测分子量 | WB,IHC,IF,IP,ELISA |
| 运输及保存条件 | Protein A |
| 宿主 | 60kD |
| 同种型 | Rabbit |
| 背景介绍 | This gene encodes a member of the chaperonin family. The encoded mitochondrial protein may function as a signaling molecule in the innate immune system. This protein is essential for the folding and assembly of newly imported proteins in the mitochondria. This gene is adjacent to a related family member and the region between the 2 genes functions as a bidirectional promoter. Several pseudogenes have been associated with this gene. Two transcript variants encoding the same protein have been identified for this gene. Mutations associated with this gene cause autosomal recessive spastic paraplegia 13. [provided by RefSeq, Jun 2010], |
| 细胞定位 | Mitochondrion matrix |
| 功能 | Disease:Defects in HSPD1 are a cause of spastic paraplegia autosomal dominant type 13 (SPG13) [MIM:605280]. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs.,Disease:Defects in HSPD1 are the cause of leukodystrophy hypomyelinating type 4 (HLD4) [MIM:612233]; also called mitochondrial HSP60 chaperonopathy or MitCHAP-60 disease. HLD4 is a severe autosomal recessive hypomyelinating leukodystrophy. Clinically characterized by infantile-onset rotary nystagmus, progressive spastic paraplegia, neurologic regression, motor impairment, profound mental retardation. Death usually occurrs within the first 2 decades of life.,Function:Implicated in mitochondrial protein import and macromolecular assembly. May facilitate the correct folding of imported proteins. May also prevent misfolding and promote the refolding and proper assembly of unfolded polypeptides generated under stress conditions in the mitochondrial matrix.,similarity:Belongs to the chaperonin (HSP60) family.,similarity:Belongs to the TCP-1 chaperonin family.,subunit:Interacts with HBV protein X and HTLV-1 protein p40tax., |
| 纯化 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Clonality | -15°C to -25°C/1 year(Do not lower than -25°C) |
| 修饰类型 | IgG,Kappa |
| 细胞定位 | Mitochondrion matrix |
| 组织表达 | Adipocyte,Adrenal gland,B-cell lymphoma,Brain,Cajal-Retzius |
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