Cell Biology Antibodies 15

Anti-HSD3B7 Antibody (CAB17777)

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SKU:
CAB17777
Product Type:
Antibody
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-HSD3B7 Antibody
Antibody SKU:CAB17777
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 120-280 of human HSD3B7 (NP_079469.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Mouse, Rat
Positive Samples:mouse brain
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 120-280 of human HSD3B7 (NP_079469.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:GTRF LVYT SSME VVGP NTKG HPFY RGNE DTPY EAVH RHPY PCSK ALAE WLVL EANG RKVR GGLP LVTC ALRP TGIY GEGH QIMR DFYR QGLR LGGW LFRA IPAS VEHG RVYV GNVA WMHV LAAR ELEQ RATL MGGQ VYFC YDGS PYRS YEDF NMEF LGPC G
Gene ID:80270
Uniprot:Q9H2F3
Cellular Location:
Calculated MW:41kDa
Observed MW:41kDa
Anti-HSD3B7 Antibody (CAB17777)Western blot - HSD3B7 Polyclonal Antibody (CAB17777)
Synonyms:CBAS1, PFIC4, SDR11E3, HSD3B7
Background:This gene encodes an enzyme which is involved in the initial stages of the synthesis of bile acids from cholesterol and a member of the short-chain dehydrogenase/reductase superfamily. The encoded protein is a membrane-associated endoplasmic reticulum protein which is active against 7-alpha hydrosylated sterol substrates. Mutations in this gene are associated with a congenital bile acid synthesis defect which leads to neonatal cholestasis, a form of progressive liver disease. Multiple transcript variants encoding different isoforms have been found for this gene.
UniProt Protein Function:HSD3B7: The 3-beta-HSD enzymatic system plays a crucial role in the biosynthesis of all classes of hormonal steroids. HSD VII is active against four 7-alpha-hydroxylated sterols. Does not metabolize several different C(19/21) steroids as substrates. Involved in bile acid synthesis. Defects in HSD3B7 are the cause of congenital bile acid synthesis defect type 1 (CBAS1); also known as neonatal progressive intrahepatic cholestasis. CBAS1 is due to a primary defect in bile synthesis leading to progressive liver disease. Clinical features include neonatal jaundice, severe intrahepatic cholestasis and cirrhosis. Belongs to the 3-beta-HSD family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Membrane protein, multi-pass; Membrane protein, integral; EC 1.1.1.181; Lipid Metabolism - primary bile acid biosynthesis; Oxidoreductase

Chromosomal Location of Human Ortholog: 16p11.2

Cellular Component: endoplasmic reticulum membrane; integral to membrane

Molecular Function:3-beta-hydroxy-delta5-steroid dehydrogenase activity; cholest-5-ene-3-beta,7-alpha-diol 3-beta-dehydrogenase activity; protein binding

Biological Process: bile acid biosynthetic process; bile acid metabolic process

Disease: Bile Acid Synthesis Defect, Congenital, 1

NCBI Summary:This gene encodes an enzyme which is involved in the initial stages of the synthesis of bile acids from cholesterol and a member of the short-chain dehydrogenase/reductase superfamily. The encoded protein is a membrane-associated endoplasmic reticulum protein which is active against 7-alpha hydrosylated sterol substrates. Mutations in this gene are associated with a congenital bile acid synthesis defect which leads to neonatal cholestasis, a form of progressive liver disease. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Dec 2008]
UniProt Code:Q9H2F3
NCBI GenInfo Identifier:47605550
NCBI Gene ID:80270
NCBI Accession:Q9H2F3.2
UniProt Secondary Accession:Q9H2F3,Q96M28, Q9BSN9,
UniProt Related Accession:Q9H2F3
Molecular Weight:21,322 Da
NCBI Full Name:3 beta-hydroxysteroid dehydrogenase type 7
NCBI Synonym Full Names:hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 7
NCBI Official Symbol:HSD3B7  
NCBI Official Synonym Symbols:CBAS1; PFIC4; SDR11E3  
NCBI Protein Information:3 beta-hydroxysteroid dehydrogenase type 7
UniProt Protein Name:3 beta-hydroxysteroid dehydrogenase type 7
UniProt Synonym Protein Names:3 beta-hydroxysteroid dehydrogenase type VII; 3-beta-HSD VII; 3-beta-hydroxy-Delta(5)-C27 steroid oxidoreductase (EC:1.1.1.-); C(27) 3-beta-HSD; Cholest-5-ene-3-beta,7-alpha-diol 3-beta-dehydrogenase (EC:1.1.1.181)
Protein Family:3 beta-hydroxysteroid dehydrogenase
UniProt Gene Name:HSD3B7  
UniProt Entry Name:3BHS7_HUMAN
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