Mouse Anti-MIPEP (AA 611-713) Recombinant Antibody (CBFYM-2236) (CBMAB-M2419-FY)

Name: Mouse Anti-MIPEP (AA 611-713) Recombinant Antibody (CBFYM-2236)
SKU: CBMAB-M2419-FY
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

CBFYM-2236

Application

ELISA, WB

Immunogen

Recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.Immunogen sequence: PYVPNTAWQL RFSHLVGYGA RYYSYLMSRA VASMVWKECF LQDPFNRAAG ERYRREMLAH GGGREPMLMV EGMLQKCPSV DDFVSALVSD LDLDFETFLM DSE

Specificity

Human

Antibody Isotype

IgG2a, k

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!]

Format

Liquid

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 611-713

More Infomation

Target

Full Name

mitochondrial intermediate peptidase

Introduction

The product of this gene performs the final step in processing a specific class of nuclear-encoded proteins targeted to the mitochondrial matrix or inner membrane. This protein is primarily involved in the maturation of oxidative phosphorylation -related proteins. This gene may contribute to the functional effects of frataxin deficiency and the clinical manifestations of Friedreich ataxia.

Entrez Gene ID

4285

UniProt ID

Q99797

Alternative Names

Mitochondrial Intermediate Peptidase; EC 3.4.24.59; MIP; COXPD31; HMIP

Function

Cleaves proteins, imported into the mitochondrion, to their mature size.

Biological Process

Peptide metabolic process Source: GO_Central
Protein processing involved in protein targeting to mitochondrion Source: GO_Central
Proteolysis Source: GO_Central

Cellular Location

Mitochondrion matrix

Involvement in disease

Combined oxidative phosphorylation deficiency 31 (COXPD31):
An autosomal recessive, severe mitochondrial disease with multisystemic manifestations appearing soon after birth or in early infancy. Clinical features include left ventricular non-compaction, global developmental delay, severe hypotonia, seizures, cataract, and abnormal movements. Death may occur in early childhood.

Tornini, V. A., Miao, L., Lee, H. J., Gerson, T., Dube, S. E., Schmidt, V., ... & Giraldez, A. J. (2023). linc-mipep and linc-wrb encode micropeptides that regulate chromatin accessibility in vertebrate-specific neural cells. Elife, 12, e82249.

Wang, L., Lu, P., Yin, J., Xu, K., Xiang, D., Zhang, Z., ... & Yang, S. (2023). Case report: Rare novel MIPEP compound heterozygous variants presenting with hypertrophic cardiomyopathy, severe lactic acidosis and hypotonia in a Chinese infant. Frontiers in Cardiovascular Medicine, 9, 1095882.

Pulman, J., Ruzzenente, B., Horak, M., Barcia, G., Boddaert, N., Munnich, A., ... & Metodiev, M. D. (2021). Variants in the MIPEP gene presenting with complex neurological phenotype without cardiomyopathy, impair OXPHOS protein maturation and lead to a reduced OXPHOS abundance in patient cells. Molecular Genetics and Metabolism, 134(3), 267-273.

Liu, J., Zhang, R., Sun, L., Zheng, Y., Chen, S., Chen, S. L., ... & Ng, T. K. (2019). Genotype–phenotype correlation and interaction of 4q25, 15q14 and MIPEP variants with myopia in southern Chinese population. British Journal of Ophthalmology.

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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)
Proteogenomics
Other Protocols

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Isotype control

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

Custom Antibody Labeling

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