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Anti-NDUFS4 Antibody (CAB8691)

SKU:
CAB8691
Product Type:
Antibody
Antibody Type:
Monoclonal Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
AQDQ
CI-18
CI-18 kDa
CI-AQDQ
Research Area:
Cell Biology
€139 - €419
Frequently bought together:

Description

Product Name:NDUFS4 Rabbit mAb
Product Code:CAB8691
Size:20uL, 50uL, 100uL
Synonyms:AQDQ, CI-18, CI-18 kDa, CI-AQDQ
Applications:WB, IHC, IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:A synthesized peptide derived from human NDUFS4
Applications:WB, IHC, IF
Recommended Dilutions:WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:Mouse liver, Mouse heart, Rat brain, Rat kidney, Rat heart, HepG2
Immunogen:A synthesized peptide derived from human NDUFS4
Purification Method:Affinity purification
Storage:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:4724
Uniprot:O43181
Cellular Location:Matrix side, Mitochondrion inner membrane, Peripheral membrane protein
Calculated MW:18kDa
Observed MW:19kDa
UniProt Protein Function:NDUFS4: Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be 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. Defects in NDUFS4 are a cause of mitochondrial complex I deficiency (MT-C1D). 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. Belongs to the complex I NDUFS4 subunit family.
UniProt Protein Details:

Protein type:EC 1.6.99.3; Oxidoreductase; EC 1.6.5.3; Mitochondrial; Energy Metabolism - oxidative phosphorylation

Chromosomal Location of Human Ortholog: 5q11.1

Cellular Component: mitochondrion; mitochondrial inner membrane; mitochondrial respiratory chain complex I

Molecular Function:NADH dehydrogenase (ubiquinone) activity

Biological Process: mitochondrial respiratory chain complex I assembly; cellular metabolic process; regulation of protein amino acid phosphorylation; cAMP-mediated signaling; positive regulation of fibroblast proliferation; response to cAMP; mitochondrial electron transport, NADH to ubiquinone; cellular respiration; brain development

Disease: Leigh Syndrome; Mitochondrial Complex I Deficiency

NCBI Summary:This gene encodes an accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), or NADH:ubiquinone oxidoreductase, the first multi-subunit enzyme complex of the mitochondrial respiratory chain. Complex I plays a vital role in cellular ATP production, the primary source of energy for many crucial processes in living cells. It removes electrons from NADH and passes them by a series of different protein-coupled redox centers to the electron acceptor ubiquinone. In well-coupled mitochondria, the electron flux leads to ATP generation via the building of a proton gradient across the inner membrane. Complex I is composed of at least 41 subunits, of which 7 are encoded by the mitochondrial genome and the remainder by nuclear genes. [provided by RefSeq, Jul 2008]
UniProt Code:O43181
NCBI GenInfo Identifier:3287881
NCBI Gene ID:4724
NCBI Accession:O43181.1
UniProt Secondary Accession:O43181,Q9BS69,
UniProt Related Accession:O43181
Molecular Weight:175
NCBI Full Name:NADH dehydrogenase
NCBI Synonym Full Names:NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase)
NCBI Official Symbol:NDUFS4  
NCBI Official Synonym Symbols:AQDQ; CI-18  
NCBI Protein Information:NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial; CI-AQDQ; CI-18 kDa; complex I-AQDQ; complex I-18 kDa; complex I 18kDa subunit; NADH-coenzyme Q reductase, 18-KD; NADH-ubiquinone oxidoreductase 18 kDa subunit; NADH dehydrogenase (ubiquinone) iron-sulfur protein 4; mitochondrial respiratory chain complex I (18-KD subunit)
UniProt Protein Name:NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial
UniProt Synonym Protein Names:Complex I-18 kDa; CI-18 kDa; Complex I-AQDQ; CI-AQDQ; NADH-ubiquinone oxidoreductase 18 kDa subunit
Protein Family:NADH dehydrogenase [ubiquinone] iron-sulfur protein
UniProt Gene Name:NDUFS4  
UniProt Entry Name:NDUS4_HUMAN

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