Metabolism Antibodies 3

Anti-ASS1 Antibody (CAB9084)

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SKU:
CAB9084
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Monoclonal Antibody
Research Area:
Metabolism

Description

system_update_altDatasheetsystem_update_altMSDS
Antibody Name:Anti-ASS1 Antibody
Antibody SKU:CAB9084
Antibody Size:20uL, 50uL, 100uL
Application:WB IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:A synthesized peptide derived from human ASS1
Application:WB IF
Recommended Dilution:WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:HeLa, Mouse testis, Mouse kidney, Rat testis, Rat kidney
Immunogen:A synthesized peptide derived from human ASS1
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:445
Uniprot:P00966
Cellular Location:
Calculated MW:47kDa
Observed MW:47KDa
Synonyms:ASS, CTLN1
Background:The protein encoded by this gene catalyzes the penultimate step of the arginine biosynthetic pathway. There are approximately 10 to 14 copies of this gene including the pseudogenes scattered across the human genome, among which the one located on chromosome 9 appears to be the only functional gene for argininosuccinate synthetase. Mutations in the chromosome 9 copy of this gene cause citrullinemia. Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Aug 2012]
UniProt Protein Function:ASS1: Defects in ASS1 are the cause of citrullinemia type 1 (CTLN1). 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. CTLN1 usually manifests in the first few days of life. Affected infants appear normal at birth, but as ammonia builds up in the body they present symptoms such as lethargy, poor feeding, vomiting, seizures and loss of consciousness. Less commonly, a milder CTLN1 form can develop later in childhood or adulthood. Belongs to the argininosuccinate synthase family. Type 1 subfamily.
UniProt Protein Details:

Protein type:Amino Acid Metabolism - alanine, aspartate and glutamate; Ligase; EC 6.3.4.5; Mitochondrial; Endoplasmic reticulum; Amino Acid Metabolism - arginine and proline

Chromosomal Location of Human Ortholog: 9q34.1

Cellular Component: cytoplasm; cytosol

Molecular Function:amino acid binding; argininosuccinate synthase activity; identical protein binding; protein binding

Biological Process: arginine biosynthetic process; argininosuccinate metabolic process; aspartate metabolic process; citrulline metabolic process; positive regulation of nitric oxide biosynthetic process; urea cycle

Disease: Citrullinemia, Classic

NCBI Summary:The protein encoded by this gene catalyzes the penultimate step of the arginine biosynthetic pathway. There are approximately 10 to 14 copies of this gene including the pseudogenes scattered across the human genome, among which the one located on chromosome 9 appears to be the only functional gene for argininosuccinate synthetase. Mutations in the chromosome 9 copy of this gene cause citrullinemia. Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Aug 2012]
UniProt Code:P00966
NCBI GenInfo Identifier:20141195
NCBI Gene ID:445
NCBI Accession:P00966.2
UniProt Secondary Accession:P00966,Q6LDL2, Q86UZ0, Q96GT4,
UniProt Related Accession:P00966
Molecular Weight:46,530 Da
NCBI Full Name:Argininosuccinate synthase
NCBI Synonym Full Names:argininosuccinate synthase 1
NCBI Official Symbol:ASS1  
NCBI Official Synonym Symbols:ASS; CTLN1  
NCBI Protein Information:argininosuccinate synthase
UniProt Protein Name:Argininosuccinate synthase
UniProt Synonym Protein Names:Citrulline--aspartate ligase
Protein Family:Assembly factor
UniProt Gene Name:ASS1  
UniProt Entry Name:ASSY_HUMAN
Anti-ASS1 Antibody (CAB9084)Western blot - ASS1 Rabbit mAb (CAB9084)
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system_update_altDatasheetHuman ASS1 Recombinant Protein Argininosuccinate Synthase (ASS1) is an urea cycle enzyme with a tetrameric structure composed of identical subunits. ASS1 is involved in