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Mouse Anti-PMPCB Recombinant Antibody (3C3) (CBMAB-P2240-YC)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
3C3
Antibody Isotype
IgG1, κ
Application
ELISA

Basic Information

Immunogen
PMPCB (NP_004270, 161-269 aa) partial recombinant protein with GST tag. Immunogen sequence: IQNSTLGEAE IERERGVILR EMQEVETNLQ EVVFDYLHAT AYQNTALGRT ILGPTENIKS ISRKDLVDYI TTHYKGPRIV LAAAGGVSHD ELLDLAKFHF GDSLCTHKG
Specificity
Human
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 161-269

Target

Full Name
peptidase (mitochondrial processing) beta
Introduction
PMPCB is a member of the peptidase M16 family and encodes a protein with a zinc-binding motif. This protein is located in the mitochondrial matrix and catalyzes the cleavage of the leader peptides of precursor proteins newly imported into the mitochondria, though it only functions as part of a heterodimeric complex.
Entrez Gene ID
9512
UniProt ID
O75439
Alternative Names
Beta-MPP; MPPB; MPPP52; MPP11; P-52
Function
Catalytic subunit of the essential mitochondrial processing protease (MPP), which cleaves the mitochondrial sequence off newly imported precursors proteins (PubMed:29576218) (Probable). Preferentially, cleaves after an arginine at position P2 (By similarity).
Required for PINK1 turnover by coupling PINK1 mitochondrial import and cleavage, which results in subsequent PINK1 proteolysis (PubMed:22354088).
Biological Process
Mitochondrial calcium ion transmembrane transportTAS:Reactome
Protein processing involved in protein targeting to mitochondrionManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Mitochondrion matrix
Involvement in disease
Multiple mitochondrial dysfunctions syndrome 6 (MMDS6):
An autosomal recessive, neurodegenerative disorder characterized by basal ganglia lesions, cerebellar atrophy, and neurologic regression in the first year of life. Common features include truncal hypotonia, lack of independent ambulation, poor speech, intellectual disability, and motor abnormalities, such as ataxia, dystonia, and spasticity.
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For research use only. Not intended for any clinical use.

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