詳細
NADH-ubiquinone oxidoreductase chain 4 (UniProt: P03905; also known as EC: 1.6.5.3, NADH dehydrogenase subunit 4, MT-ND4) is encoded by the MT-ND4 (also known as MTND4, NADH4, ND4) gene (Gene ID: 4538) in human. MT-ND4 is an inner mitochondrial membrane protein that belongs to the complex I subunit 4 family. It is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I). The complex couples the oxidation of NADH and the reduction of ubiquinone (Coenzyme Q10), to the generation of a proton gradient, which is then used for ATP synthesis. The complex can be dissociated into two main sub-complexes, corresponding to the "ankle" of the boot, and the "foot" of the boot. The ankle is thought to protrude from the membrane so as to be predominantly in the aqueous phase on the matrix side. It contains the binding site for NAD(H), and the input electron transfer chain. The foot (hydrophobic) is membrane bound and contains a catalytic site at which ubiquinone is reduced. Defects in MT-ND4 gene are known to cause Leber hereditary optic neuropathy (LHON) that is characterized by acute or subacute loss of central vision due to optic nerve dysfunction. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. Other diseases associated with MT-ND4 gene mutations are age-related macular degeneration, mesial temporal lobe epilepsy, and cystic fibrosis.
特異性
Clone 9E4-2D8 detects NADH-ubiquinone oxidoreductase chain 4 in mitochodria isolated from human cells.
免疫原
KLH-conjugated linear peptide from the C-terminal region.
アプリケーション
Anti-MT-ND4, clone 9E4-2D8, Cat. No. MABS1994,is a mouse monoclonal antibody that detects NADH-ubiquinone oxidoreductase chain 4 and has been tested for use in Western Blotting.
Western Blotting Analysis: 4 µg/mL from a representative lot detected MT-ND4 in mitochondria from Human Neonatal Dermal Fibroblasts and mitochondria from Human neonatal dermal fibroblasts depleted of mtDNA. (Courtesy of Michael F. Marusich, Ph.D., mAbDx, Inc., Eugene, OR, USA).
品質
Evaluated by Western Blotting in Mitochondria from human neonatal dermal fibroblasts and mitochondria from human neonatal dermal fibroblasts depleted of mtDNA.
Western Blotting Analysis: 1 µg/mL of this antibody detected MT-ND4 in Mitochondria from human neonatal dermal fibroblasts and did not detect it in mitochondria from human neonatal dermal fibroblasts depleted of mtDNA.
ターゲットの説明
~37 kDa observed; 51.58 kDa calculated. Uncharacterized bands may be observed in some lysate(s).
物理的形状
Format: Purified
Purified mouse monoclonal antibody IgG2a in buffer containing HEPES-Buffered Saline (150 mM NaCl, 15 mM HEPES, pH 7.2) with 0.02% sodium azide.
その他情報
Concentration: Please refer to lot specific datasheet.