烟酰胺核苷酸转氢酶抗体
规格:1mg/1ml
英文名: NNT
别名: NAD(P) transhydrogenase mitochondrial; nicotinamide nucleotide transhydrogenase; NNTM; Pyridine nucleotide transhydrogenase; NNTM_HUMAN.
分子量: 109kDa
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 NNT
交叉反应:Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,
烟酰胺核苷酸转氢酶抗体细胞定位:细胞浆 细胞膜 线粒体
产品介绍:background: Nicotinamide nucleotide transhydrogenase (NNT) is an integral protein of the inner mitochondrial membrane.It couples hydride transfer between NAD(H) and NADP(+) to proton translocation across the inner mitochondrial membrane. Function: The transhydrogenation between NADH and NADP is coupled to respiration and ATP hydrolysis and functions as a proton pump across the membrane. May play a role in reactive oxygen species (ROS) detoxification in the adrenal gland. Subunit: Homodimer Subcellular Location: Mitochondrion inner membrane; Multi-pass membrane protein; Matrix side Tissue Specificity: Widely expressed with expression most readily detectable in adrenal, heart, kidney, thyroid and adipose tissues. DISEASE: Defects in NNT are the cause of glucocorticoid deficiency type 4 (GCCD4) [MIM:614736]. A rare, potentially lethal, autosomal recessive disorder characterized by resistance to ACTH action on the adrenal cortex, adrenal insufficiency烟酰胺核苷酸转氢酶抗体 and an inability of the adrenal cortex to produce cortisol. It usually presents in the neonatal period or in early childhood with episodes of hypoglycemia and other symptoms related to cortisol deficiency, including failure to thrive, recurrent illnesses or infections, convulsions, and shock. In a small number of patients hypoglycemia can be sufficiently severe and persistent that it leads to serious long-term neurological damage or death. The diagnosis is readily confirmed with a low plasma cortisol measurement in the presence of an elevated ACTH level, and normal aldosterone and plasma renin measurements. Similarity: In the N-terminal section; belongs to the AlaDH/PNT family. In the C-terminal section; belongs烟酰胺核苷酸转氢酶抗体 to the PNT beta subunit family. Gene ID: 23530 Database links: Entrez Gene: 280878 Cow Entrez Gene: 23530 Human SwissProt: P11024 Cow SwissProt: Q13423 Human SwissProt: Q61941 Mouse Unigene: 5120 Cow Unigene: 482043 Human Unigene: 195803 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
烟酰胺核苷酸转氢酶抗体产品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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 **学 转录调节因子 线粒体
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid