Anti-ASS1 Antibody (CAB2012)
- SKU:
- CAB2012
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Metabolism
Frequently bought together:
Description
| Antibody Name: | Anti-ASS1 Antibody |
| Antibody SKU: | CAB2012 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IF |
| 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 IF |
| Recommended Dilution: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | LO2, HeLa, Mouse heart, Mouse kidney, Rat kidney |
| 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 |
| 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 |
 | Western blot analysis of extracts of various cell lines, using ASS1 antibody (CAB2012) 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: 1s. |
 | Immunofluorescence analysis of HeLa cells using ASS1 Rabbit pAb (CAB2012) at dilution of 1:100. Blue: DAPI for nuclear staining. |
 | Immunofluorescence analysis of NIH/3T3 cells using ASS1 Rabbit pAb (CAB2012) at dilution of 1:100. Blue: DAPI for nuclear staining. |
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