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Rabbit Anti-SLC25A13 Recombinant Antibody (BA0384) (CBMAB-0849CQ)


See all SLC25A13 antibodies
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Summary

Host Animal
Rabbit
Specificity
Human
Clone
BA0384
Antibody Isotype
IgG
Application
WB

Basic Information

Immunogen
Human SLC25A13 (C terminal)
Specificity
Human
Antibody Isotype
IgG
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!]

Purity
>95% as determined by analysis by SDS-PAGE
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 Member 13
Introduction
This gene is a member of the mitochondrial carrier family. Catalyzes the calcium-dependent exchange of cytoplasmic glutamate with mitochondrial aspartate across the mitochondrial inner membrane. May have a function in the urea cycle.
Entrez Gene ID
10165
UniProt ID
Q9UJS0
Alternative Names
CTLN2; CITRIN; ARALAR2
Function
Mitochondrial electrogenic aspartate/glutamate antiporter that favors efflux of aspartate and entry of glutamate and proton within the mitochondria as part of the malate-aspartate shuttle (PubMed:11566871).
Also mediates the uptake of L-cysteinesulfinate by mitochondria in exchange of L-glutamate and proton. Can also exchange L-cysteinesulfinate with aspartate in their anionic form without any proton translocation (PubMed:11566871).
Biological Process
Biological Process aspartate family amino acid metabolic processTAS:Reactome
Biological Process aspartate transmembrane transportManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process ATP biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process cellular respirationManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process gluconeogenesisTAS:Reactome
Biological Process L-glutamate transmembrane transportManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process malate-aspartate shuttleManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process mitochondrial transport1 PublicationNAS:UniProtKB
Biological Process response to calcium ionManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Citrullinemia 2 (CTLN2):
A form of citrullinemia, an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. Citrullinemia type 2 is characterized by neuropsychiatric symptoms including abnormal behaviors, loss of memory, seizures and coma. Death can result from brain edema. Onset is sudden and usually between the ages of 20 and 50 years.
Cholestasis, neonatal intrahepatic, caused by citrin deficiency (NICCD):
A form of citrullinemia type 2 with neonatal onset, characterized by suppression of the bile flow, hepatic fibrosis, low birth weight, growth retardation, hypoproteinemia, variable liver dysfunction. Neonatal intrahepatic cholestasis due to citrin deficiency is generally not severe and symptoms disappear by one year of age with an appropriate diet. Years or even decades later, however, some individuals develop the characteristic features of citrullinemia type 2 with neuropsychiatric symptoms.
Topology
Mitochondrial intermembrane: 2-331
Helical: 332-349
Mitochondrial matrix: 350-392
Helical: 393-412
Mitochondrial intermembrane: 413-435
Helical: 436-449
Mitochondrial matrix: 450-484
Helical: 485-504
Mitochondrial intermembrane: 505-523
Helical: 524-541
Mitochondrial matrix: 542-580
Helical: 581-600
Mitochondrial intermembrane: 601-675
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For research use only. Not intended for any clinical use.

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