核苷酸结合蛋白样NUBPL抗体
规格：1mg/1ml
英文名： NUBPL
别名： C14orf127; FLJ12660; huInd1; IND1 homolog; Iron-sulfur protein NUBPL; Nubpl; NUBPL_HUMAN; nucleotide binding protein like; Nucleotide-binding protein-like.
分子量： 34kDa
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 NUBPL
交叉反应：Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Guinea Pig, Cat,
细胞定位：
核苷酸结合蛋白样NUBPL抗体产品介绍：background: This gene encodes a member of the Mrp/NBP35 ATP-binding proteins family. The encoded protein is required for the assembly of the respiratory chain NADH dehydrogenase (complex I), an oligomeric enzymatic complex located in the inner mitochondrial membrane. Mutations in this gene cause mitochondrial complex I deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, May 2014] Function: Required for the assembly of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I). May deliver of one or more Fe-S clusters to complex I subunits. Subcellular Location: Mitochondrion. Tissue Specificity: Highest expression in liver and kidney. expressed at significant levels in small intestine and brain (at protein level). DISEASE: Defects in NUBPL are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes 核苷酸结合蛋白样NUBPL抗体a wide range of clinical manifestations from lethal neonatal disease to *****-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. Similarity: Belongs to the Mrp/NBP35 ATP-binding proteins 核苷酸结合蛋白样NUBPL抗体family. Database links: Entrez Gene: 80224 Human Entrez Gene: 76826 Mouse Entrez Gene: 299008 Rat Omim: 613621 Human SwissProt: Q8TB37 Human SwissProt: Q9CWD8 Mouse Unigene: 288981 Human Unigene: 244781 Mouse Unigene: 13455 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
核苷酸结合蛋白样NUBPL抗体产品应用：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：细胞生物 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid