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

Name: Mouse Anti-PMPCB Recombinant Antibody (3C3)
SKU: CBMAB-P2240-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

3C3

Application

ELISA

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

More Infomation

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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