NDUFAF6蛋白抗体
规格：1mg/1ml
英文名： NDUFAF6
别名： CCDC113; C8orf38; NDUF6_HUMAN; Coiled-coil domain-containing protein 113 Gene names; DKFZp434N1418; HSPC065; NADH dehydrogenase (ubiquinone) complex I assembly factor 6; Putative phytoene synthase; UP
分子量： 33kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human NDUFAF6
交叉反应：Human, Mouse, Rat,
细胞定位：细胞核 细胞浆
NDUFAF6蛋白抗体产品介绍：background: This gene encodes a protein that localizes to mitochondria and contains a predicted phytoene synthase domain. The encoded protein plays an important role in the assembly of complex I (NADH-ubiquinone oxidoreductase) of the mitochondrial respiratory chain through regulation of subunit ND1 biogenesis. Mutations in this gene are associated with complex I enzymatic deficiency. [provided by RefSeq, Nov 2011] Function: Involved in the assembly of mitochondrial NADH:ubiquinone oxidoreductase complex (complex I) at early stages. May play a role in the biogenesis of MT-ND1. Subcellular Location: Isoform 1: Mitochondrion inner membrane. Note: Peripherally localized on the matrix face of the mitochondrial inner membrane. Ref.7 Isoform 2: Cytoplasm. Nucleus DISEASE: Mitochondrial complex I deficiency (MT-C1D) [MIM:252010]: A disorder of the mitochondrial respiratory chain that causes a wide range of clinical manifestations from lethal neonatal disease to *****-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathyNDUFAF6蛋白抗体, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy,NDUFAF6蛋白抗体 and some forms of Parkinson disease. Note: The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the NDUFAF6 family. Gene ID: 137682 Database links: Entrez Gene: 137682 Human Entrez Gene: 523017 Cow Entrez Gene: 76947 Mouse Entrez Gene: 297821 Rat Omim: 612392 Human SwissProt: A7YVD7 Cow SwissProt: Q330K2 Human SwissProt: A2AIL4 Mouse SwissProt: D3ZN43 Rat Unigene: 729144 Human Important Note: This product as supplied is intended for NDUFAF6蛋白抗体research use only, not for use in human, therapeutic or diagnostic applications.
产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 神经生物学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid