人 PITRM1 抗体对
01AB01297
Pitrilysin metallopeptidase 1 (PITRM1), also known as presequence protease (PreP), is a 117 kDa ATP-dependent zinc metallopeptidase located in the mitochondrial matrix, where it plays a crucial role in mitochondrial proteostasis by degrading post-cleavage mitochondrial targeting sequences and a broad range of unstructured peptides, including amyloid beta (Aβ) fragments. Structurally, PITRM1 consists of two homologous domains (hPreP-N and hPreP-C) connected by a hinge region, forming a large catalytic chamber that enables size-exclusion-based substrate selection, allowing degradation of peptides between 10 and 65 amino acids but not larger folded proteins. The enzyme is essential for mitochondrial function, as it prevents the accumulation of toxic peptides that could impair mitochondrial activity. Clinically, PITRM1 deficiency has been linked to neurodegenerative diseases such as Alzheimer's disease, due to impaired degradation of mitochondrial Aβ, and is associated with conditions like spinocerebellar ataxia, autosomal recessive 30, and mitochondrial DNA depletion syndrome 7.
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技术参数
- 靶点基因名
- PITRM1
- 基因别名
- KIAA1104; MP1; PREP
- 蛋白别名
- pitrilysin metallopeptidase 1; PITRM1
- UniProt
- Q5JRX3
- 物种
- Human
产品详情
- 靶点名称
- PITRM1
- 蛋白名称
- pitrilysin metallopeptidase 1
- 蛋白别名
- Presequence protease; mitochondrial; hPreP; Pitrilysin metalloproteinase 1; Metalloprotease 1; hMP1
- 靶点基因名
- PITRM1
- 基因别名
- KIAA1104; MP1; PREP
- UniProt
- Q5JRX3
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Pitrilysin metallopeptidase 1 (PITRM1), also known as presequence protease (PreP), is a 117 kDa ATP-dependent zinc metallopeptidase located in the mitochondrial matrix, where it plays a crucial role in mitochondrial proteostasis by degrading post-cleavage mitochondrial targeting sequences and a broad range of unstructured peptides, including amyloid beta (Aβ) fragments. Structurally, PITRM1 consists of two homologous domains (hPreP-N and hPreP-C) connected by a hinge region, forming a large catalytic chamber that enables size-exclusion-based substrate selection, allowing degradation of peptides between 10 and 65 amino acids but not larger folded proteins. The enzyme is essential for mitochondrial function, as it prevents the accumulation of toxic peptides that could impair mitochondrial activity. Clinically, PITRM1 deficiency has been linked to neurodegenerative diseases such as Alzheimer's disease, due to impaired degradation of mitochondrial Aβ, and is associated with conditions like spinocerebellar ataxia, autosomal recessive 30, and mitochondrial DNA depletion syndrome 7.