Home > Product > Antibody > Rabbit Anti-MT-ND3 antibody
Mitochondrially encoded NADH dehydrogenase 3; MT-ND3; NADH dehydrogenase subunit 3; NADH-ubiquinone oxidoreductase chain 3; ND3; NU3M_HUMAN.
Cat:
SL17867R
Species Reactivity:
(predicted: Human,)
Immunogen:
KLH conjugated synthetic peptide derived from human MT-ND3:1-80/115
Format:
Liquid
Storage instructions:
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500ICC=1:100-500IF=1:100-500(Paraffin sections need to do antigen repair)not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
More
Unit:
Price: $
Product PDFs
Datasheet:


NADH:ubiquinone oxidoreductase (complex I) is an extremely complicated multiprotein complex located in the inner mitochondrial membrane. Human complex I is important for energy metabolism because its main function is to transport electrons from NADH to ubiquinone, which is accompanied by trans-location of protons from the mitochondrial matrix to the intermembrane space. Human complex I appears to consist of 41 subunits. A small number of complex I subunits are the products of mitochondrial genes (subunits 1-7), while the remainder are nuclear encoded and imported from the cytoplasm. NADH dehydrogenase subunit 3 (ND3) localizes to the hydrophobic protein fragment of complex I. Mutations in the gene encodiing for ND3 may be associated with Parkinson disease.

Function:
Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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.

Subcellular Location:
Mitochondrion membrane.

DISEASE:
Defects in MT-ND3 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.
Defects in MT-ND3 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, 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.

Similarity:
Belongs to the complex I subunit 3 family.

SWISS:
P03897

Gene ID:
4537

Database links:

Entrez Gene: 4537 Human

Entrez Gene: 17718 Mouse

Entrez Gene: 26199 Rat

SwissProt: P03897 Human

SwissProt: P03899 Mouse

SwissProt: P05506 Rat




Product Feedback Wall
Message :
Your Email :
Copyright © 2007-2018 Sunlong Medical All Rights Reserved.