HSPD1 Protein (His-GST)
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Quick Overview for HSPD1 Protein (His-GST) (ABIN7317578)
抗原
See all HSPD1 蛋白蛋白类型
宿主
资源
纯度
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标记
- This HSPD1 protein is labelled with His-GST.
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原理
- Recombinant Human HSPD1/HSP60 Protein (His & GST Tag)
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序列
- Leu 2-Phe 573
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产品特性
- A DNA sequence encoding the human HSP60 (NP_955472.1) (Leu 2-Phe 573) was fused with the N-terminal polyhistidine-tagged GST tag at the N-terminus.
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过滤
- 0.2 μm filtered
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Biological Activity Comment
- Not validated for activity
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限制
- 仅限研究用
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状态
- Lyophilized
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缓冲液
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Lyophilized from sterile PBS, pH 7.4
Normally 5 % - 8 % trehalose, mannitol and 0.01 % Tween 80 are added as protectants before lyophilization. -
储存条件
- 4 °C,-20 °C,-80 °C
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储存方法
- Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.
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有效期
- 12 months
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- HSPD1 (Heat Shock 60kDa Protein 1 (Chaperonin) (HSPD1))
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别名
- HSPD1/HSP60
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背景
- CPN60,GROEL,HLD4,HSP-60,HSP60,HSP65,HuCHA60,SPG13,HSPD1, also known as HSP60, is a member of the chaperonin family. HSPD1 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. It may also prevent misfolding and promote the refolding and proper assembly of unfolded polypeptides generated under stress conditions in the mitochondrial matrix. HSPD1 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. Mutations associated with this gene cause autosomal recessive spastic paraplegia 13.Defects in HSPD1 are a cause of spastic paraplegia autosomal dominant type 13 (SPG13). Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Defects in HSPD1 are the cause of leukodystrophy hypomyelinating type 4 (HLD4), also called mitochondrial HSP60 chaperonopathy or MitCHAP-60 disease. HLD4 is a severe autosomal recessive hypomyelinating leukodystrophy. HSPD1 is cinically characterized by infantile-onset rotary nystagmus, progressive spastic paraplegia, neurologic regression, motor impairment, profound mental retardation. Death usually occurrs within the first two decades of life.
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分子量
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Calculated MW: 88.7 kDa
Observed MW: 52-65 kDa
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NCBI登录号
- NP_955472
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UniProt
- A0A024R3X4
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途径
- Activation of Innate immune Response, Regulation of Leukocyte Mediated Immunity, Positive Regulation of Immune Effector Process, Production of Molecular Mediator of Immune Response, Positive Regulation of Endopeptidase Activity
抗原
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