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Code
OSS00283W
$240USD
ID Tag
Rb1526-130810-WS
Immunogen
A synthetic peptide from n-terminal region of human SLC25A15 conjugated to blue carrier protein was used as the antigen.
Accession
Also known
Mitochondrial ornithine transporter 1, Solute carrier family 25 member 15, ORNT1, SP1855
Target
FUNCTION: 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.
SUBCELLULAR LOCATION: Mitochondrion inner membrane; Multi-pass membrane protein
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.
SUBCELLULAR LOCATION: Mitochondrion inner membrane; Multi-pass membrane protein
Storage
Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
Expiry Date
12 months after reconstitution
Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.
References
1. Camacho J.A, et al. Nat. Genet. 22:151-158(1999)
2. Fiermonte G, et al. J. Biol. Chem. 278:32778-32783(2003)
3. Tessa A, et al. Hum. Mutat. 30:741-748(2009)
2. Fiermonte G, et al. J. Biol. Chem. 278:32778-32783(2003)
3. Tessa A, et al. Hum. Mutat. 30:741-748(2009)
Limitation
For research use only
Code
OSS00283W
$240USD
Unit size
100 µl
Conjugate
Unconjugated antibody
Host
NZ white rabbit
Purity
Whole serum
Clonality
Polyclonal
Isotype
Polyclonal, whole serum
Applications
IHC, WB (confirmed by recombinant protein). A dilution of 1: 200 to 1: 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specificity
Specific for SLC25A15.
Spcs X-react.
Human. Other species not yet tested.
Format
Lyophilised
Reconstitution
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.
Note
Control peptide is available at $120 per 50 μg. Please enquire sales@osenses.com
Code
OSS00283W
$240USD
PDF Data Sheet
- Click to download the Data SheetMSDS
⚬ Products
Rabbit antibody to SLC25A15
