Mouse Anti-ACADSB Recombinant Antibody (V2-179180) (CBMAB-A0437-YC)

Name: Mouse Anti-ACADSB Recombinant Antibody (V2-179180)
SKU: CBMAB-A0437-YC
Rating: 5 (1 reviews)

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Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

V2-179180

Antibody Isotype

IgG1, κ

Application

WB, IP, IF, ELISA

Basic Information

Immunogen

Raised against amino acids 368-411 mapping near the C-terminus of ACADSB of human origin

Specificity

Human

Antibody Isotype

IgG1, κ

Clonality

Monoclonal

Application Notes

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

Application	Note
WB	1:100-1:1,000
IP	1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
IF(ICC)	1:50-1:500
ELISA	1:30-1:3,000

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

Buffer

PBS, 0.1% gelatin

Preservative

< 0.1% sodium azide

Concentration

0.2 mg/ml

Storage

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

Target

Full Name

Acyl-CoA Dehydrogenase Short/Branched Chain

Introduction

Short/branched chain acyl-CoA dehydrogenase(ACADSB) is a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. Substrate specificity

Entrez Gene ID

UniProt ID

P45954

Alternative Names

Acyl-CoA Dehydrogenase Short/Branched Chain; Acyl-Coenzyme A Dehydrogenase, Short/Branched Chain; 2-Methyl Branched Chain Acyl-CoA Dehydrogenase; 2-Methylbutyryl-Coenzyme A Dehydrogenase; 2-MEBCAD; SBCAD;

Function

Short and branched chain specific acyl-CoA dehydrogenase that catalyzes the removal of one hydrogen from C-2 and C-3 of the fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, acts specifically on short and branched chain acyl-CoA derivatives such as (S)-2-methylbutyryl-CoA as well as short straight chain acyl-CoAs such as butyryl-CoA. Plays an important role in the metabolism of L-isoleucine by catalyzing the dehydrogenation of 2-methylbutyryl-CoA, one of the steps of the L-isoleucine catabolic pathway. Can also act on valproyl-CoA, a metabolite of valproic acid, an antiepileptic drug

Biological Process

Branched-chain amino acid catabolic process
Fatty acid metabolic process
Isoleucine catabolic process

Cellular Location

Mitochondrion matrix

Involvement in disease

Autosomal recessive disorder and consists of a defect in catabolism of L-isoleucine which is characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Affected individuals have seizures and psychomotor delay as the main clinical features.

More Infomation

References

Lu, D., Yang, Z., Xia, Q., Gao, S., Sun, S., Luo, X., ... & Li, X. (2020). ACADSB regulates ferroptosis and affects the migration, invasion, and proliferation of colorectal cancer cells. Cell Biology International, 44(11), 2334-2343.

Lin, Y., Gao, H., Lin, C., Chen, Y., Zhou, S., Lin, W., ... & Fu, Q. (2019). Biochemical, clinical, and genetic characteristics of short/Branched chain acyl-CoA dehydrogenase deficiency in Chinese patients by newborn screening. Frontiers in genetics, 10, 802.

Porta, F., Chiesa, N., Martinelli, D., & Spada, M. (2019). Clinical, biochemical, and molecular spectrum of short/branched-chain acyl-CoA dehydrogenase deficiency: two new cases and review of literature. Journal of Pediatric Endocrinology and Metabolism, 32(2), 101-108.

Jiang, P., Fang, X., Zhao, Z., Yu, X., Sun, B., Yu, H., & Yang, R. (2018). The effect of short/branched chain acyl-coenzyme A dehydrogenase gene on triglyceride synthesis of bovine mammary epithelial cells. Archives Animal Breeding, 61(1), 115-122.

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Protocols

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)
Proteogenomics
Other Protocols

Associated Products

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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Documents

Datasheet
SDS
Request for COA

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Second antibody and isotype control

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