Anti-ASS1 Antibody (CAB16767)[KO Validated]

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SKU:
CAB16767
Product type:
Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism
Frequently bought together:

Description

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Antibody Name:Anti-ASS1 Antibody [KO Validated]
Antibody SKU:CAB16767
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-412 of human ASS1 (NP_446464.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:HeLa
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-412 of human ASS1 (NP_446464.1).
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:MSSK GSVV LAYS GGLD TSCI LVWL KEQG YDVI AYLA NIGQ KEDF EEAR KKAL KLGA KKVF IEDV SREF VEEF IWPA IQSS ALYE DRYL LGTS LARP CIAR KQVE IAQR EGAK YVSH GATG KGND QVRF ELSC YSLA PQIK VIAP WRMP EFYN RFKG RNDL MEYA KQHG IPIP VTPK NPWS MDEN LMHI SYEA GILE NPKN QAPP GLYT KTQD PAKA PNTP DILE IEFK KGVP VKVT NVKD GTTH QTSL ELFM YLNE VAGK HGVG RIDI VENR FIGM KSRG IYET PAGT ILYH AHLD IEAF TMDR EVRK IKQG LGLK FAEL VYTG FWHS PECE FVRH CIAK SQER VEGK VQVS VLKG QVYI LGRE SPLS LYNE ELVS MNVQ GDYE PTDA TGFI NINS LRLK EYHR LQSK VTAK
Gene ID:445
Uniprot:P00966
Cellular Location:
Calculated MW:46kDa
Observed MW:46kDa
Anti-ASS1 Antibody [KO Validated] (CAB16767)Western blot analysis of extracts of HeLa cells, using ASS1 Rabbit pAb (CAB16767) 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.
Anti-ASS1 Antibody [KO Validated] (CAB16767)Western blot analysis of extracts from normal (control) and ASS1 Rabbit pAb knockout (KO) HeLa cells, using ASS1 Rabbit pAb antibody (CAB16767) 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

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