SLC25A13, Polyclonal Antibody

CTLN2; CITRIN; ARALAR2

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

The antibody detects endogenous levels of total SLC25A13 protein.

Antigen Affinity Purification

Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% Glycerol.

1.9 mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-SLC25A13 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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 isoformshave been found for this gene.

Total protein Ab

Western Blot (WB), Immunohistochemistry (IHC)

Western Blot: 1:500-1:2000
Immunohistochemistry: 1:25-1:100

Gel: 8%SDS-PAGE Lysate: 40 μ g Lane 1-3: Hepg2 cells, A172 cells, Raji cells Primary antibody: 1/450 dilution Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution Exposure time: 1 minute

Immunohistochemical analysis of paraffin-embedded Human thyroid cancer tissue using at dilution 1/35.

Immunohistochemical analysis of paraffin-embedded Human liver cancer tissue using at dilution 1/35.

74,304 Da

solute carrier family 25 member 13

calcium-binding mitochondrial carrier protein Aralar2

Calcium-binding mitochondrial carrier protein Aralar2

ARALAR2  [Similar Products]

CMC2_HUMAN

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.

Protein type: Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial; Transporter; Transporter, SLC family

Chromosomal Location of Human Ortholog: 7q21.3

Cellular Component: mitochondrial inner membrane; mitochondrion

Molecular Function: acidic amino acid transmembrane transporter activity; calcium ion binding; L-aspartate transmembrane transporter activity; L-glutamate transmembrane transporter activity

Biological Process: aspartate transport; ATP biosynthetic process; cellular respiration; gluconeogenesis; L-glutamate transport; malate-aspartate shuttle; response to calcium ion

Disease: Citrullinemia, Type Ii, Adult-onset; Citrullinemia, Type Ii, Neonatal-onset

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