Code
OSS00292W
$240USD
ID Tag
Rb1615-120910-WS
Immunogen
A synthetic peptide from c-terminal region of human Mitochondrial glutamate carrier 1 (SLC25A22) conjugated to an immunogenic carrier protein was used as the antigen.
Accession
Also known
GC-1, Glutamate/H(+) symporter 1, Solute carrier family 25 member 22, SLC25A22, GC1
Target
FUNCTION: Involved in the transport of glutamate across the inner mitochondrial membrane. Glutamate is cotransported with H
+.
Defects in SLC25A22 are the cause of epileptic encephalopathy early infantile type 3 (EIEE3)
; also known as early myoclonic encephalopathy (EME) or neonatal epilepsy with suppression-burst pattern. Severe neonatal epilepsies with suppression-burst pattern are early-onset epileptic syndromes characterized by a typical EEG pattern. The suppression-burst pattern of the EEG is characterized by higher-voltage bursts of slow waves mixed with multifocal spikes alternating with isoelectric suppression phases. EME is characterized by a very early onset, erratic and fragmentary myoclonus, massive myoclonus, partial motor seizures and late tonic spasms. The prognosis of EME is poor, with no effective treatment, and children with the condition either die within 1 to 2 years after birth or survive in a persistent vegetative state. EME inheritance is autosomal recessive.
SUBCELLULAR LOCATION: Mitochondrion inner membrane; Multi-pass membrane protein
Tissue specificity: Highly expressed in most tissues.
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. Fiermonte G, et al. J. Biol. Chem. 277:19289-19294(2002)
2. Molinari F, et al. Am. J. Hum. Genet. 76:334-339(2005)
Limitation
For research use only
Code
OSS00292W
$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 SLC25A22.
Spcs X-react.
Human. Other species not yet tested but expected to work in rat and mouse.
Format
Lyophilised
Reconstitution
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.