Rabbit Anti-HSD3B7 Recombinant Antibody (EG1562) (CBMAB-EN1864-LY)

Name: Rabbit Anti-HSD3B7 Recombinant Antibody (EG1562)
SKU: CBMAB-EN1864-LY
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Rabbit

Clone

EG1562

Application

WB: 1:500~1:1000 ELISA: 1:20000

Immunogen

The antibody was produced against synthesized peptide derived from internal of human HSD3B7.

Specificity

Human, Mouse, Rat

Antibody Isotype

IgG

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

More Infomation

Target

Full Name

hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 7

Introduction

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.

Entrez Gene ID

Human	80270
Mouse	101502
Rat	246211

UniProt ID

Human	Q9H2F3
Mouse	Q9EQC1
Rat	O35048

Alternative Names

CBAS1; PFIC4; SDR11E3

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 (PubMed:11067870).

Plays a key role in cell positioning and movement in lymphoid tissues by mediating degradation of 7-alpha,25-dihydroxycholesterol (7-alpha,25-OHC): 7-alpha,25-OHC acts as a ligand for the G protein-coupled receptor GPR183/EBI2, a chemotactic receptor for a number of lymphoid cells.

Biological Process

B cell chemotaxis Source: UniProtKB
Bile acid biosynthetic process Source: UniProtKB

Cellular Location

Endoplasmic reticulum membrane

Involvement in disease

Congenital bile acid synthesis defect 1 (CBAS1):
A primary defect in bile synthesis leading to progressive liver disease. Clinical features include neonatal jaundice, severe intrahepatic cholestasis, cirrhosis.

Zou, C., Wang, W., Shu, C., Liang, S., Zou, Y., Wang, L., ... & You, F. (2023). Expression characteristics of Hsd3b7 in the gonads of Paralichthys olivaceus. Comparative Biochemistry and Physiology Part B: Biochemistry and Molecular Biology, 266, 110848.

Jebaying, Y., Kumar, K., Malhotra, S., & Sibal, A. (2023). Novel mutation in the HSD3B7 gene causes bile acid synthetic disorder and presents as recurrent liver failure in early childhood. BMJ Case Reports CP, 16(2), e245852.

Panda, K., Dash, M., Bisoi, S. K., Mallick, B., & Jena, N. (2023). Bile Acid Synthetic Disorder Manifesting as Hepatomegaly And Reversible Coagulopathy In A 3-Year-Old Child: Discovery Of A Novel Mutation In HSD3B7 Gene. Journal of Clinical and Experimental Hepatology, 13, S119.

Hu, Z., Wang, J., Fu, Y., Li, Y., & Jiang, Q. (2022). HSD3B7 as a prognostic-related biomarker predicts poor prognostic in ccRCC.

Zhao, J., Setchell, K. D., Gong, Y., Sun, Y., Zhang, P., Heubi, J. E., ... & Wang, J. S. (2021). Genetic spectrum and clinical characteristics of 3β-hydroxy-Δ5-C27-steroid oxidoreductase (HSD3B7) deficiency in China. Orphanet Journal of Rare Diseases, 16(1), 1-15.

Lu, Z. J., Wang, L., Sun, X. Y., Li, J. Y., Cheng, L., Li, N. N., & Peng, R. (2018). The association between HSD3B7 gene variant and Parkinson's disease in ethnic Chinese. Brain and Behavior, 8(4), e00913.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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