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Anti-NNT Antibody (CAB4561)

SKU:
CAB4561
Product Type:
Antibody
Antibody Type:
Polyclonal Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
NNT
GCCD4
Research Area:
Cell Biology
  • Cell Biology Antibodies 9 Anti-NNT Antibody CAB4561
  • Cell Biology Antibodies 9 Anti-NNT Antibody CAB4561
$167 - $503
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Description

Product Name:NNT Rabbit pAb
Product Code:CAB4561
Size:20uL, 50uL, 100uL
Synonyms:NNT, GCCD4
Applications:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 881-1086 of human NNT (NP_036475.3).
Applications:WB
Recommended Dilutions:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:HepG2, Mouse heart, Mouse liver, Rat kidney
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 881-1086 of human NNT (NP_036475.3).
Purification Method:Affinity purification
Storage:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:NRSL ANVI LGGY GTTS TAGG KPME ISGT HTEI NLDN AIDM IREA NSII ITPG YGLC AAKA QYPI ADLV KMLT EQGK KVRF GIHP VAGR MPGQ LNVL LAEA GVPY DIVL EMDE INHD FPDT DLVL VIGA NDTV NSAA QEDP NSII AGMP VLEV WKSK QVIV MKRS LGVG YAAV DNPI FYKP NTAM LLGD AKKT CDAL QAKV RESY QK
Gene ID:23530
Uniprot:Q13423
Cellular Location:Matrix side, Mitochondrion inner membrane, Multi-pass membrane protein
Calculated MW:113kDa
Observed MW:114kDa/125kDa
UniProt Protein Function:NNT: 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. Defects in NNT are the cause of glucocorticoid deficiency type 4 (GCCD4). 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.
UniProt Protein Details:

Protein type:Cofactor and Vitamin Metabolism - nicotinate and nicotinamide; Mitochondrial; EC 1.6.1.2; Membrane protein, integral; Oxidoreductase; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 5p12

Cellular Component: mitochondrion; membrane; mitochondrial inner membrane; integral to membrane; mitochondrial respiratory chain

Molecular Function:NAD(P) transhydrogenase activity; NAD(P)+ transhydrogenase (AB-specific) activity; NADP binding; NAD binding; NAD(P)+ transhydrogenase (B-specific) activity

Biological Process: cellular metabolic process; proton transport; cell redox homeostasis; tricarboxylic acid cycle

Disease: Glucocorticoid Deficiency 4

NCBI Summary:This gene encodes an integral protein of the inner mitochondrial membrane. The enzyme couples hydride transfer between NAD(H) and NADP(+) to proton translocation across the inner mitochondrial membrane. Under most physiological conditions, the enzyme uses energy from the mitochondrial proton gradient to produce high concentrations of NADPH. The resulting NADPH is used for biosynthesis and in free radical detoxification. Two alternatively spliced variants, encoding the same protein, have been found for this gene. [provided by RefSeq, Jul 2008]
UniProt Code:Q13423
NCBI GenInfo Identifier:51338801
NCBI Gene ID:23530
NCBI Accession:Q13423.3
UniProt Secondary Accession:Q13423,Q16796, Q2TB60, Q8N3V4,
UniProt Related Accession:Q13423
Molecular Weight:1086
NCBI Full Name:NAD(P) transhydrogenase, mitochondrial
NCBI Synonym Full Names:nicotinamide nucleotide transhydrogenase
NCBI Official Symbol:NNT  
NCBI Official Synonym Symbols:GCCD4  
NCBI Protein Information:NAD(P) transhydrogenase, mitochondrial; NAD(P) transhydrogenase, mitochondrial; pyridine nucleotide transhydrogenase
UniProt Protein Name:NAD(P) transhydrogenase, mitochondrial
UniProt Synonym Protein Names:Nicotinamide nucleotide transhydrogenase; Pyridine nucleotide transhydrogenase
Protein Family:NAD(P) transhydrogenase
UniProt Gene Name:NNT  
UniProt Entry Name:NNTM_HUMAN

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