SLC25A13 Antibody (PACO43611)
제품 코드를 사용하여 Assay Genie 메인 사이트를 통해 주문합니다.
주문- SKU:
- PACO43611
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
Antibody Name: | SLC25A13 Antibody (PACO43611) |
Antibody SKU: | PACO43611 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:500-1:2000, IHC:1:20-1:200 |
Species Reactivity: | Human, Mouse |
Immunogen: | Recombinant Human Calcium-binding mitochondrial carrier protein Aralar2 protein (1-300AA) |
Form: | Liquid |
Storage Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Purification Method: | Antigen Affinity Purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Western blot. All lanes: Calcium-binding mitochondrial carrier protein Aralar2 antibody at 4µg/ml. Lane 1: Mouse liver tissue. Lane 2: Mouse kidney tissue. Lane 2: HepG2 whole cell lysate. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 74 kDa. Observed band size: 74 kDa. | |
Immunohistochemistry of paraffin-embedded human colon cancer using PACO43611 at dilution of 1:100. |
Background: | Catalyzes the calcium-dependent exchange of cytoplasmic glutamate with mitochondrial aspartate across the mitochondrial inner membrane. May have a function in the urea cycle. |
Synonyms: | Calcium-binding mitochondrial carrier protein Aralar2 (Citrin) (Mitochondrial aspartate glutamate carrier 2) (Solute carrier family 25 member 13), SLC25A13, ARALAR2 |
UniProt Protein Function: | SLC25A13: Catalyzes the calcium-dependent exchange of cytoplasmic glutamate with mitochondrial aspartate across the mitochondrial inner membrane. May have a function in the urea cycle. Defects in SLC25A13 are the cause of citrullinemia type 2 (CTLN2). 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). 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. Belongs to the mitochondrial carrier family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Mitochondrial; Membrane protein, multi-pass; Membrane protein, integral; Transporter, SLC family; Transporter Chromosomal Location of Human Ortholog: 7q21.3 Cellular Component: mitochondrion; integral to plasma membrane; mitochondrial inner membrane Molecular Function:L-aspartate transmembrane transporter activity; transporter activity; calcium ion binding; L-glutamate transmembrane transporter activity Biological Process: L-glutamate transport; transport; ATP biosynthetic process; carbohydrate metabolic process; cellular respiration; glucose metabolic process; pathogenesis; aspartate transport; malate-aspartate shuttle; response to calcium ion; gluconeogenesis Disease: Citrullinemia, Type Ii, Adult-onset; Citrullinemia, Type Ii, Neonatal-onset |
NCBI Summary: | This gene is a member of the mitochondrial carrier family. The encoded protein contains four EF-hand Ca(2+) binding motifs in the N-terminal domain, and localizes to mitochondria. The protein 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 this gene result in citrullinemia, type II. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2009] |
UniProt Code: | Q9UJS0 |
NCBI GenInfo Identifier: | 13124095 |
NCBI Gene ID: | 10165 |
NCBI Accession: | Q9UJS0.2 |
UniProt Related Accession: | Q9UJS0 |
Molecular Weight: | |
NCBI Full Name: | Calcium-binding mitochondrial carrier protein Aralar2 |
NCBI Synonym Full Names: | solute carrier family 25 member 13 |
NCBI Official Symbol: | SLC25A13 |
NCBI Official Synonym Symbols: | CTLN2; CITRIN; ARALAR2 |
NCBI Protein Information: | calcium-binding mitochondrial carrier protein Aralar2 |
UniProt Protein Name: | Calcium-binding mitochondrial carrier protein Aralar2 |
UniProt Synonym Protein Names: | Citrin; Mitochondrial aspartate glutamate carrier 2; Solute carrier family 25 member 13 |
Protein Family: | COX assembly mitochondrial protein |
UniProt Gene Name: | SLC25A13 |
UniProt Entry Name: | CMC2_HUMAN |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
Recommended Products |
Anti-FITC Goat Anti-Rabbit IgG (H+L) Antibody (CABS011) |
Anti-HRP-conjugated Beta Actin Antibody (CABC028) |