Mouse Anti-TIMMDC1 Recombinant Antibody (CBYJT-3190) (CBMAB-T2464-YJ)

Name: Mouse Anti-TIMMDC1 Recombinant Antibody (CBYJT-3190)
SKU: CBMAB-T2464-YJ
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

CBYJT-3190

Application

WB, IP, IF, ELISA

Specificity

Human

Antibody Isotype

IgG

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!]

Buffer

PBS, pH 7.4

Storage

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

More Infomation

Target

Full Name

Translocase Of Inner Mitochondrial Membrane Domain Containing 1

Introduction

Diseases associated with TIMMDC1 include Mitochondrial Complex I Deficiency. Among its related pathways are Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins and metabolism.

Entrez Gene ID

51300

UniProt ID

Q9NPL8

Alternative Names

Translocase Of Inner Mitochondrial Membrane Domain Containing 1; Translocase Of Inner Mitochondrial Membrane Domain-Containing Protein 1; TIMM Domain Containing-Protein 1; C3orf1; Complex I Assembly Factor TIMMDC1, Mitochondrial; Chromosome 3 Open Reading Frame 1; Transmembrane Protein C3orf1; M5-14 Protein; Protein M5-14

Function

Chaperone protein involved in the assembly of the mitochondrial NADH:ubiquinone oxidoreductase complex (complex I). Participates in constructing the membrane arm of complex I.

Cellular Location

Mitochondrion membrane

Involvement in disease

Mitochondrial complex I deficiency, nuclear type 31 (MC1DN31):
A form of mitochondrial complex I deficiency, the most common biochemical signature of mitochondrial disorders, a group of highly heterogeneous conditions characterized by defective oxidative phosphorylation, which collectively affects 1 in 5-10000 live births. Clinical disorders have variable severity, ranging from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. MC1DN31 transmission pattern is consistent with autosomal recessive inheritance.

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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

Related Products

Mouse Anti-TIMMDC1 Recombinant Antibody (CBYJT-3189) (CAT#: CBMAB-T2463-YJ)

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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