Rabbit anti-Human, Mouse ASS Polyclonal Antibody | anti-ASS antibody

ASS, ID (Argininosuccinate Synthase, Citrulline-aspartate Ligase, ASS1) (HRP)

Cat. Num. AAA6463949

• Gene Names: ASS1; ASS; CTLN1
• Reactivity: Human, Mouse
• Applications: Western Blot, ELISA
• Purity: Purified by Ammonium Sulfate Precipitation.
• Synonyms: ASS; Polyclonal Antibody; ID (Argininosuccinate Synthase; Citrulline-aspartate Ligase; ASS1) (HRP); anti-ASS antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human ASS. Species Crossreactivity: mouse.
• Purity/Purification: Purified by Ammonium Sulfate Precipitation.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Horseradish Peroxidase (HRP).

• Applicable Applications for anti-ASS antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:50-1:100; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the center region of human ASS (KLH).
• Conjugate: HRP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Note: Sodium azide is a potent inhibitor of peroxidase and should not be added to HRP conjugates.

Western Blot (WB)

(Western blot analysis of ASS Antibody (Center) in mouse kidney tissue lysates (35ug/lane). ASS (arrow) was detected using the purified Pab.)

Western Blot (WB)

(Western blot analysis of ASS Antibody (Center) in Hela cell line lysates (35ug/lane). ASS (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human colon carcinoma reacted with ASS Antibody (Center), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)

Flow Cytometry (FC/FACS)

(Flow cytometric analysis of widr cells using ASS Antibody (Center)(bottom histogram) compared to a negative control cell (top histogram)FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Immunofluorescence (IF)

(Confocal immunofluorescent analysis of ASS Antibody (Center) with Hela cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green).DAPI was used to stain the cell nuclear (blue).)

Product Categories/Family for anti-ASS antibody

Antibodies; Enzymes; Synthase

References

1. Engel, K., et.al., Hum. Mutat. 30(3), 300-307 (2009).

NCBI and Uniprot Product Information

• NCBI GI #: 53759107
• NCBI GeneID: 445
• NCBI Accession #: NP_000041.2
• NCBI GenBank Nucleotide #: NM_000050.4
• UniProt Accession #: P00966; Q6LDL2; Q86UZ0; Q96GT4
• Molecular Weight: 46,530 Da
• NCBI Official Full Name: argininosuccinate synthase
• NCBI Official Synonym Full Names: argininosuccinate synthase 1
• NCBI Official Symbol: ASS1
• NCBI Official Synonym Symbols: ASS; CTLN1
• NCBI Protein Information: argininosuccinate synthase; argininosuccinate synthetase 1; citrulline--aspartate ligase; citrulline-aspartate ligase
• UniProt Protein Name: Argininosuccinate synthase
• Protein Family: Assembly factor
• UniProt Gene Name: ASS1
• UniProt Synonym Gene Names: ASS
• UniProt Entry Name: ASSY_HUMAN

NCBI Description

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 Description

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.

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

Chromosomal Location of Human Ortholog: 9q34.1

Cellular Component: mitochondrial outer membrane; lysosome; endoplasmic reticulum; cytoplasm; perikaryon; nucleus; cytosol

Molecular Function: amino acid binding; toxin binding; protein binding; argininosuccinate synthase activity; ATP binding

Biological Process: response to drug; positive regulation of nitric oxide biosynthetic process; arginine biosynthetic process; response to mycotoxin; citrulline metabolic process; liver development; response to estradiol stimulus; response to zinc ion; acute-phase response; midgut development; aspartate metabolic process; kidney development; argininosuccinate metabolic process; response to nutrient; urea cycle; aging

Disease: Citrullinemia, Classic