Main Information

Target

NDUFA1

Host Species

Rabbit

Reactivity

Human, Mouse, Rat

Applications

IHC, ELISA

Conjugate/Modification

Unmodified

Detailed Information

Recommended Dilution Ratio

IHC 1:50-200; ELISA(peptide)1:5000-20000

Formulation

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Specificity

This antibody detects endogenous levels of human NDUFA1

Purification

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Storage

-15°C to -25°C/1 year(Do not lower than -25°C)

Concentration

1 mg/ml

Modification

Unmodified

Clonality

Polyclonal

Isotype

IgG

Antigen&Target Information

Immunogen:

Synthesized peptide derived from human NDUFA1 AA range: 20-100

Specificity:

This antibody detects endogenous levels of human NDUFA1

Gene Name:

NDUFA1

Protein Name:

NDUFA1

Other Name:

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 ;
Complex I-MWFE ;
CI-MWFE ;
NADH-ubiquinone oxidoreductase MWFE subunit ;

Database Link:

Organism	Gene ID	SwissProt
Human	4694;	O15239;

Background:

The human NDUFA1 gene codes for an essential component of complex I of the respiratory chain, which transfers electrons from NADH to ubiquinone. It has been noted that the N-terminal hydrophobic domain has the potential to be folded into an alpha-helix spanning the inner mitochondrial membrane with a C-terminal hydrophilic domain interacting with globular subunits of complex I. The highly conserved two-domain structure suggests that this feature is critical for the protein function and might act as an anchor for the NADH:ubiquinone oxidoreductase complex at the inner mitochondrial membrane. However, the NDUFA1 peptide is one of about 31 components of the "hydrophobic protein" (HP) fraction of complex I which is involved in proton translocation. Thus the NDUFA1 peptide may also participate in that function. [provided by RefSeq, Jul 2008],

Function:

Disease:Defects in NDUFA1 are a cause of complex I mitochondrial respiratory chain deficiency [MIM:252010]. Complex I (NADH-ubiquinone oxidoreductase), the largest complex of the mitochondrial respiratory chain, contains more than 40 subunits. It is embedded in the inner mitochondrial membrane and is partly protruding in the matrix. Complex I deficiency is the most common cause of mitochondrial disorders. It represents largely one-third of all cases of respiratory chain deficiency and is responsible for a variety of clinical symptoms, ranging from neurological disorders to cardiomyopathy, liver failure, and myopathy.,Function:Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed to be not involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.,similarity:Belongs to the complex I NDUFA1 subunit family.,subunit:Complex I is composed of 45 different subunits.,tissue specificity:Primarily expressed in heart and skeletal muscle.,

Cellular Localization:

Mitochondrion inner membrane ; Single-pass membrane protein ; Matrix side .

Tissue Expression:

Primarily expressed in heart and skeletal muscle.

Research Areas:

>>Oxidative phosphorylation ;
>>Metabolic pathways ;
>>Thermogenesis ;
>>Retrograde endocannabinoid signaling ;
>>Non-alcoholic fatty liver disease ;
>>Alzheimer disease ;
>>Parkinson disease ;
>>Amyotrophic lateral sclerosis ;
>>Huntington disease ;
>>Prion disease ;
>>Pathways of neurodegeneration - multiple diseases ;
>>Chemical carcinogenesis - reactive oxygen species ;
>>Diabetic cardiomyopathy