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SLC25A13 Polyclonal Antibody
Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | Q9UJS0 |
| Reactivity | Rat, Pig, Dog, Bovine |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 74 KDa |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human SLC25A13 |
| Epitope Specificity | 351-450/675 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Mitochondrion inner membrane; Multi-pass membrane protein. |
| SIMILARITY | Belongs to the mitochondrial carrier family. Contains 4 EF-hand domains. Contains 3 Solcar repeats. |
| DISEASE | Defects in SLC25A13 are the cause of citrullinemia type 2 (CTLN2) [MIM:603471]. Citrullinemia belongs to the urea cycle disorders. It is an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. CTLN2 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. Defects in SLC25A13 are the cause of neonatal intrahepatic cholestasis due to citrin deficiency (NICCD) [MIM:605814]. NICCD is a form of citrullinemia type 2 with neonatal onset. NICCD is characterized by suppression of the bile flow, hepatic fibrosis, low birth weight, growth retardation, hypoproteinemia, variable liver dysfunction. NICCD 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | SLC25A13 is a member of the mitochondrial carrier family. It contains four EF-hand Ca(2+) binding motifs in the N-terminal domain, and localizes to mitochondria. It catalyzes the exchange of aspartate for glutamate and a proton across the inner mitochondrial membrane, and is stimulated by calcium on the external side of the inner mitochondrial membrane. Mutations in the SLC25A13 gene result in citrullinemia, type II. Multiple transcript variants encoding different isoforms have been found for this gene. |
| Gene ID | 10165 |
|---|---|
| Other Names | Calcium-binding mitochondrial carrier protein Aralar2, Citrin, Mitochondrial aspartate glutamate carrier 2, Solute carrier family 25 member 13, SLC25A13, ARALAR2 |
| Target/Specificity | High levels in liver and low levels in kidney, pancreas, placenta, heart and brain. |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| Format | 0.01M TBS(pH7.4), 0.09% (W/V) sodium azide and 50% Glyce |
| Storage | Store at -20 ℃ for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 ℃. |
| Name | SLC25A13 (HGNC:10983) |
|---|---|
| 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, PubMed:38945283). Also mediates the uptake of L- cysteinesulfinate (3-sulfino-L-alanine) by mitochondria in exchange of L-glutamate and proton (PubMed:11566871). Can also exchange L- cysteinesulfinate with aspartate in their anionic form without any proton translocation (PubMed:11566871). Lacks transport activity towards gamma-aminobutyric acid (GABA) (PubMed:38945283). |
| Cellular Location | Mitochondrion inner membrane; Multi-pass membrane protein |
| Tissue Location | High levels in liver and low levels in kidney, pancreas, placenta, heart and brain. |
Research Areas
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$ 385.00
Cat# AP58119
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