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Product Name HSD3B7 Chinese Name 滋养层细胞抗原3β7抗体 Alias 3 beta HSD VII; 3 beta hydroxy Delta(5) C27 steroid oxidoreductase; 3 beta hydroxysteroid dehydrogenase type 7; 3 beta hydroxysteroid dehydrogenase type VII; C(27) 3 beta HSD; Cca2; Cholest 5 ene 3 beta,7 alpha diol 3 beta dehydrogenase; Confluent 3Y1 cell associated 2; Hydroxy delta 5 steroid dehydrogenase, 3 beta and steroid delta isomerase 7; OTTMUSP00000018894; PFIC4; SDR11E3; BB098564; OTTMUSP00000018895; OTTMUSP00000018897; AI195443. Research Area Tumour Cell biology immunology endothelial cells Immunogen Species Rabbit Clonality Polyclonal React Species Human, Rat, (predicted: Mouse, Pig, Cow, Rabbit, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 41kDa Cellular localization cytoplasmic The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human HSD3B7: 281-369/369 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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].
Function:
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.
Subcellular Location:
Endoplasmic reticulum membrane; Multi-pass membrane protein.
Tissue Specificity:
High levels in liver and lung, moderate levels in spleen, brain, heart, kidney, jejunum and testis. Up-regulated in 3Y1 cells upon growth arrest.
DISEASE:
Defects in HSD3B7 are the cause of congenital bile acid synthesis defect type 1 (CBAS1) [MIM:607765]; 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.
Similarity:
Belongs to the 3-beta-HSD family.
SWISS:
Q9H2F3
Gene ID:
80270
Database links:Entrez Gene: 80270 Human
Entrez Gene: 101502 Mouse
Omim: 607764 Human
SwissProt: Q9H2F3 Human
SwissProt: Q9EQC1 Mouse
Product Picture Sample:
Lane 1: Human HeLa cell lysates
Lane 2: Human HepG2 cell lysates
Lane 3: Human MCF-7 cell lysates
Lane 4: Human A549 cell lysates
Primary: Anti-HSD3B7 (SL2366R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 41 kDa
Observed band size: 45 kDa
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