Product Name	Anti-NNT/RBITC
Chinese Name	罗丹明（RBITC）标记的烟酰胺核苷酸转氢酶抗体
Alias	NAD(P) transhydrogenase mitochondrial; nicotinamide nucleotide transhydrogenase; NNTM; Pyridine nucleotide transhydrogenase; NNTM_HUMAN.
Research Area	Cell biology  immunology  transcriptional regulatory factor  Mitochondrion
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human, Dog,  (predicted: Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit, )
Applications	IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	109kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human NNT
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	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.
Product Detail	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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