NDUFS1 Antibody (PACO10794)
- SKU:
- PACO10794
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
抗体名: | NDUFS1 Antibody (PACO10794) |
抗体コード: | PACO10794 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC, IF |
推奨される希釈: | |
反応性: | Human, Mouse, Rat |
免疫原: | Human NDUFS1 |
憲法: | Liquid |
ストレージバッファ: | PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
精製方法: | Antigen Affinity Purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
シノニム: | NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase);NDUFS1;CI-75Kd;MGC26839;PRO1304 ; |
UniProt Protein Function: | NDUFS1: 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. This is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Defects in NDUFS1 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 75 kDa subunit family. 4 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Mitochondrial; EC 1.6.99.3; EC 1.6.5.3; Oxidoreductase; Energy Metabolism - oxidative phosphorylation Chromosomal Location of Human Ortholog: 2q33-q34 Cellular Component: mitochondrion; mitochondrial inner membrane; mitochondrial intermembrane space; mitochondrial respiratory chain complex I Molecular Function:2 iron, 2 sulfur cluster binding; protein binding; NADH dehydrogenase (ubiquinone) activity; electron carrier activity; metal ion binding; 4 iron, 4 sulfur cluster binding Biological Process: ATP metabolic process; cellular metabolic process; apoptotic mitochondrial changes; regulation of mitochondrial membrane potential; mitochondrial electron transport, NADH to ubiquinone; cellular respiration Disease: Mitochondrial Complex I Deficiency |
NCBI Summary: | The protein encoded by this gene belongs to the complex I 75 kDa subunit family. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. This protein is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Mutations in this gene are associated with complex I deficiency. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jan 2011] |
UniProt Code: | P28331 |
NCBI GenInfo Identifier: | 92090799 |
NCBI Gene ID: | 4719 |
NCBI Accession: | P28331.3 |
UniProt Secondary Accession: | P28331,Q53TR8, Q8N1C4, Q8TCC9, B4DIN9, B4DJA0, B4DPG1 B4DUC1, E7ENF3, |
UniProt Related Accession: | P28331 |
Molecular Weight: | 727 |
NCBI Full Name: | NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial |
NCBI Synonym Full Names: | NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase) |
NCBI Official Symbol: | NDUFS1 |
NCBI Official Synonym Symbols: | CI-75k; CI-75Kd; PRO1304 |
NCBI Protein Information: | NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial; complex I 75kDa subunit; complex I, mitochondrial respiratory chain, 75-kD subunit; mitochondrial NADH-ubiquinone oxidoreductase 75 kDa subunit |
UniProt Protein Name: | NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial |
UniProt Synonym Protein Names: | Complex I-75kD; CI-75kD |
UniProt Gene Name: | NDUFS1 |
UniProt Entry Name: | NDUS1_HUMAN |