Name: Anti-ASS1 Antibody (CAB19878)[KO Validated]
Brand: KO Validated Antibodies 2
SKU: CAB19878
Availability: OutOfStock

Antibody Name:	Anti-ASS1 Antibody [KO Validated]
Antibody SKU:	CAB19878
Antibody Size:	20uL, 50uL, 100uL
Application:	WB
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant protein of human ASS1.

Application:	WB
Recommended Dilution:	WB 1:500 - 1:2000
Reactivity:	Human, Mouse, Rat
Positive Samples:	HeLa

Immunogen:	Recombinant protein of human ASS1.
Purification Method:	Affinity purification
Storage Buffer:	Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	Email for sequence
Gene ID:	445
Uniprot:	P00966
Cellular Location:
Calculated MW:	46kDa
Observed MW:	46KDa

Anti-ASS1 Antibody [KO Validated] (CAB19878)

Western blot analysis of extracts from normal (control) and ASS1 knockout (KO) HeLa cells, using ASS1 antibody (CAB19878) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 5s.

Synonyms:

ASS1, 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.

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

Antibodies	ELISA Kits
Anti-ASS1 Antibody (CAB2012)	Human ASS1 / CTLN1 / Argininosuccinate synthase ELISA Kit
Anti-ASS1 Antibody (CAB16767)[KO Validated]	Human Argininosuccinate synthase (ASS1) ELISA Kit
Anti-ASS1 Antibody (CAB9084)
ASS1 Antibody (PACO07892)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)