Western blot
All lanes: PITRM1 antibody at 2µg/ml
Lane 1: A549 whole cell lysate
Lane 2: Mouse kidney tissue
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 118, 107 kDa
Observed band size: 118 kDa
Immunohistochemistry of paraffin-embedded human brain tissue using CSB-PA692283ESR1HU at dilution of 1:100
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貨期:
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用途:
For Research Use Only. Not for use in diagnostic or therapeutic procedures.
Metalloendopeptidase of the mitochondrial matrix that functions in peptide cleavage and degradation rather than in protein processing. Has an ATP-independent activity. Specifically cleaves peptides in the range of 5 to 65 residues. Shows a preference for cleavage after small polar residues and before basic residues, but without any positional preference. Degrades the transit peptides of mitochondrial proteins after their cleavage. Also degrades other unstructured peptides. It is also able to degrade amyloid-beta protein 40, one of the peptides produced by APP processing, when it accumulates in mitochondrion. It is a highly efficient protease, at least toward amyloid-beta protein 40. Cleaves that peptide at a specific position and is probably not processive, releasing digested peptides intermediates that can be further cleaved subsequently.
基因功能參考文獻:
Redox control of hPreP in the mitochondrial matrix and the protective role of the conserved methionine 206 residue as an internal antioxidant. PMID: 23041349
The authors identified two siblings carrying a homozygous PITRM1 missense mutation (c.548G>A, p.Arg183Gln) associated with an autosomal recessive, slowly progressive syndrome characterised by mental retardation, spinocerebellar ataxia, cognitive decline and psychosis. PMID: 26697887
Data show that the hPreP presequence only targets GFP to the matrix of mammalian and yeast mitochondria. PMID: 21621546
This study demonstrated decreased proteolytic activity of the mitochondrial amyloid-beta degrading enzyme, PreP peptidasome, in Alzheimer's disease brain mitochondria. PMID: 21750375
our data suggest that genetic variation in the hPreP gene PITRM1 may potentially contribute to mitochondrial dysfunctions PMID: 19962426
The substrate specificity of the mitochondrial metallopeptidase proteinase 1 (MP1) was investigated and its mitochondrial targeting signal identified. PMID: 19196155
Data revealed that six polymorphisms of F10, PITRM1, PCSK2, JPH3, MYO7B, and AKAP12 were related (P<0.05) to the prevalence of chronic kidney disease. PMID: 19724895
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亞細胞定位:
Mitochondrion matrix.
蛋白家族:
Peptidase M16 family, PreP subfamily
組織特異性:
Widely expressed. Expressed at higher level in muscle and heart compared to brain, pancreas, liver, lung and placenta.