NDUFA1 Antibody, HRP conjugated (PACO28747)
- SKU:
- PACO28747
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- HRP
Frequently bought together:
Description
抗体名: | NDUFA1 Antibody, HRP conjugated (PACO28747) |
抗体コード: | PACO28747 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human NADH dehydrogenase [ubiquinone] 1 α subcomplex subunit 1 protein (1-70AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | HRP |
バックグラウンド: | NDUFA1 (NADH dehydrogenase (ubiquinone) 1 α subcomplex, 1) is an essential component of the multisubunit NADH ubiquinone oxidoreductase (complex 1), the first enzyme complex in the mitochondrial respiratory chain. Complex I transfers electrons from NADH to the respiratory chain via ubiquinone. |
シノニム: | NADH dehydrogenase [ubiquinone] 1 α subcomplex subunit 1 (Complex I-MWFE) (CI-MWFE) (NADH-ubiquinone oxidoreductase MWFE subunit), NDUFA1 |
UniProt Protein Function: | NDUFA1: 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 NDUFA1 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 NDUFA1 subunit family. |
UniProt Protein Details: | Protein type:Oxidoreductase; Mitochondrial; EC 1.6.5.3; Membrane protein, integral; Energy Metabolism - oxidative phosphorylation; EC 1.6.99.3 Chromosomal Location of Human Ortholog: Xq24 Cellular Component: mitochondrion; mitochondrial inner membrane; mitochondrial membrane; integral to membrane; mitochondrial respiratory chain complex I Molecular Function:NADH dehydrogenase (ubiquinone) activity Biological Process: cellular metabolic process; mitochondrial electron transport, NADH to ubiquinone Disease: Mitochondrial Complex I Deficiency |
NCBI Summary: | 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] |
UniProt Code: | O15239 |
NCBI GenInfo Identifier: | 3334271 |
NCBI Gene ID: | 4694 |
NCBI Accession: | O15239.1 |
UniProt Related Accession: | O15239 |
Molecular Weight: | 8,072 Da |
NCBI Full Name: | NADH dehydrogenase |
NCBI Synonym Full Names: | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa |
NCBI Official Symbol: | NDUFA1 |
NCBI Official Synonym Symbols: | MWFE; ZNF183; CI-MWFE |
NCBI Protein Information: | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1; complex I-MWFE; type I dehydrogenase; complex I MWFE subunit; NADH oxidoreductase subunit MWFE; NADH:ubiquinone oxidoreductase (com |
UniProt Protein Name: | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 |
UniProt Synonym Protein Names: | Complex I-MWFE; CI-MWFE; NADH-ubiquinone oxidoreductase MWFE subunit |
UniProt Gene Name: | NDUFA1 |
UniProt Entry Name: | NDUA1_HUMAN |