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Mouse Anti-SLC25A20 Recombinant Antibody (CBXS-0441) (CBMAB-S3391-CQ)

This product is a mouse antibody that recognizes SLC25A20. The antibody CBXS-0441 can be used for immunoassay techniques such as: WB, ICC, IHC-P, IHC-Fr, ELISA.
See all SLC25A20 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBXS-0441
Application
WB, ICC, IHC-P, IHC-Fr, ELISA

Basic Information

Immunogen
Carnitine Acylcarnitine Translocase
Specificity
Human
Clonality
Monoclonal
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
Buffer
PBS, pH 7.4, 50% glycerol
Preservative
0.02% sodium azide
Concentration
0.5 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
solute carrier family 25 (carnitine/acylcarnitine translocase), member 20
Introduction
This gene product is one of several closely related mitochondrial-membrane carrier proteins that shuttle substrates between cytosol and the intramitochondrial matrix space. This protein mediates the transport of acylcarnitines into mitochondrial matrix for their oxidation by the mitochondrial fatty acid-oxidation pathway. Mutations in this gene are associated with carnitine-acylcarnitine translocase deficiency, which can cause a variety of pathological conditions such as hypoglycemia, cardiac arrest, hepatomegaly, hepatic dysfunction and muscle weakness, and is usually lethal in new born and infants.
Entrez Gene ID
UniProt ID
Alternative Names
Solute Carrier Family 25 Member 20; Solute Carrier Family 25 (Carnitine/Acylcarnitine Translocase), Member 20; Carnitine/Acylcarnitine Translocase; CACT; CAC; Mitochondrial Carnitine/Acylcarnitine Carrier Protein; Carnitine-Acylcarnitine Carrier;
Function
Mediates the electroneutral exchange of acylcarnitines (O-acyl-(R)-carnitine or L-acylcarnitine) of different acyl chain lengths (ranging from O-acetyl-(R)-carnitine to long-chain O-acyl-(R)-carnitines) with free carnitine ((R)-carnitine or L-carnitine) across the mitochondrial inner membrane, via a ping-pong mechanism (PubMed:12892634, PubMed:18307102) (Probable). Key player in the mitochondrial oxidation pathway, it translocates the fatty acids in the form of acylcarnitines into the mitochondrial matrix, where the carnitine palmitoyltransferase 2 (CPT-2) activates them to undergo fatty acid beta-oxidation (Probable). Catalyzes the unidirectional transport (uniport) of carnitine at lower rates than the antiport (exchange) (PubMed:18307102).
Biological Process
Biological Process carnitine shuttleTAS:Reactome
Biological Process carnitine transmembrane transportISS:UniProtKB
Biological Process in utero embryonic developmentIEA:Ensembl
Biological Process mitochondrial transportManual Assertion Based On ExperimentIBA:GO_Central
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Carnitine-acylcarnitine translocase deficiency (CACTD):
A rare long-chain fatty acid oxidation disorder. Metabolic consequences include hypoketotic hypoglycemia under fasting conditions, hyperammonemia, elevated creatine kinase and transaminases, dicarboxylic aciduria, very low free carnitine and abnormal acylcarnitine profile with marked elevation of the long-chain acylcarnitines. Clinical features include neurologic abnormalities, cardiomyopathy, arrhythmias, skeletal muscle damage, liver dysfunction and episodes of life-threatening coma, which eventually lead to death. Most patients become symptomatic in the neonatal period with a rapidly progressive deterioration and a high mortality rate.
Topology
Cytoplasmic: 2-12
Helical: 13-31
Mitochondrial matrix: 32-73
Helical: 74-93
Cytoplasmic: 94-112
Helical: 113-131
Mitochondrial matrix: 132-170
Helical: 171-190
Cytoplasmic: 191-211
Helical: 212-230
Mitochondrial matrix: 231-267
Helical: 268-287
Cytoplasmic: 288-301
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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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