SLC25A15, Polyclonal Antibody

HHH; ORC1; ORNT1; D13S327

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

Polyclonal

IgG

Rabbit

Affinity Purification

1mg/ml (lot specific)

Store at -20 degree C (regular) or -80 degree C (long term). Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Small volumes of anti-SLC25A15 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 transports ornithine across the inner mitochondrial membrane from the cytosol to the mitochondrial matrix. The protein is an essential component of the urea cycle, and functions in ammonium detoxification and biosynthesis of the amino acid arginine. Mutations in this gene result in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome. There is a pseudogene of this locus on the Y chromosome.

Polyclonal

Western Blot (WB), Immunohistochemistry (IHC)

WB: 1:200-1:2000
IHC: 1:50-1:200

Western blot analysis of extracts of various cell lines, using SLC25A15 antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Enhanced Kit.
Exposure time: 30s.

301

solute carrier family 25 (mitochondrial carrier; ornithine transporter) member 15

mitochondrial ornithine transporter 1; solute carrier family 25 member 15

Mitochondrial ornithine transporter 1

ORNT1  [Similar Products]

ORNT1_HUMAN

This gene is a member of the mitochondrial carrier family. The encoded protein transports ornithine across the inner mitochondrial membrane from the cytosol to the mitochondrial matrix. The protein is an essential component of the urea cycle, and functions in ammonium detoxification and biosynthesis of the amino acid arginine. Mutations in this gene result in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome. There is a pseudogene of this locus on the Y chromosome.[provided by RefSeq, May 2009]

SLC25A15: Ornithine transport across inner mitochondrial membrane, from the cytoplasm to the matrix. Defects in SLC25A15 are the cause of hyperornithinemia- hyperammonemia-homocitrullinuria syndrome (HHH syndrome). It is an autosomal recessive disorder resulting in various neurologic symptoms, including mental retardation, spastic paraparesis with pyramidal signs, cerebellar ataxia, and episodic disturbance of consciousness or coma caused by hyperammonemia. It causes a functional impairment of the urea cycle. Belongs to the mitochondrial carrier family.

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

Chromosomal Location of Human Ortholog: 13q14

Cellular Component: mitochondrial inner membrane; integral to membrane

Molecular Function: L-ornithine transmembrane transporter activity

Biological Process: amino acid metabolic process; mitochondrial ornithine transport; urea cycle

Disease: Hyperornithinemia-hyperammonemia-homocitrullinuria Syndrome

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