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Rabbit Anti-NNT/PE-Cy7 Conjugated antibody (bs-5097R-PE-Cy7)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-5097R-PE-Cy7
英文名称 Rabbit Anti-NNT/PE-Cy7 Conjugated antibody
中文名称 PE-Cy7标记的烟酰胺核苷酸转氢酶抗体
别    名 NAD(P) transhydrogenase mitochondrial; nicotinamide nucleotide transhydrogenase; NNTM; Pyridine nucleotide transhydrogenase; NNTM_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  转录调节因子  线粒体  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Dog,  (predicted: Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit, )
产品应用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 109kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NNT
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
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 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 to the PNT beta subunit family.

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.
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