Anti-PTS Antibody (CAB6306)
- SKU:
- CAB6306
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
| Antibody Name: | Anti-PTS Antibody |
| Antibody SKU: | CAB6306 |
| 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 1-145 of human PTS (NP_000308.1). |
| Application: | WB IF |
| Recommended Dilution: | WB 1:500 - 1:2000 IF 1:10 - 1:100 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | HeLa, U-251MG, Mouse liver, Mouse kidney |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-145 of human PTS (NP_000308.1). |
| 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: | MSTE GGGR RCQA QVSR RISF SASH RLYS KFLS DEEN LKLF GKCN NPNG HGHN YKVV VTVH GEID PATG MVMN LADL KKYM EEAI MQPL DHKN LDMD VPYF ADVV STTE NVAV YIWD NLQK VLPV GVLY KVKV YETD NNIV VYKG E |
| Gene ID: | 5805 |
| Uniprot: | Q03393 |
| Cellular Location: | |
| Calculated MW: | 16kDa |
| Observed MW: | 16kDa |
| Synonyms: | PTS, PTPS |
| Background: | The enzyme encoded by this gene catalyzes the elimination of inorganic triphosphate from dihydroneopterin triphosphate, which is the second and irreversible step in the biosynthesis of tetrahydrobiopterin from GTP. Tetrahydrobiopterin, also known as BH(4), is an essential cofactor and regulator of various enzyme activities, including enzymes involved in serotonin biosynthesis and NO synthase activity. Mutations in this gene result in hyperphenylalaninemia. |
| UniProt Protein Function: | PTS: Involved in the biosynthesis of tetrahydrobiopterin, an essential cofactor of aromatic amino acid hydroxylases. Catalyzes the transformation of 7,8-dihydroneopterin triphosphate into 6- pyruvoyl tetrahydropterin. Defects in PTS are the cause of BH4-deficient hyperphenylalaninemia type A (HPABH4A); also called 6-pyruvoyl-tetrahydropterin synthase deficiency (PTS deficiency) or hyperphenylalaninemia tetrahydrobiopterin-deficient due to PTS deficiency. HPABH4A is an autosomal recessive disorder characterized by depletion of the neurotransmitters dopamine and serotonin, and clinically by severe neurological symptoms unresponsive to the classic phenylalanine-low diet. Belongs to the PTPS family. |
| UniProt Protein Details: | Protein type:Mitochondrial; Cofactor and Vitamin Metabolism - folate biosynthesis; Lyase; EC 4.2.3.12 Chromosomal Location of Human Ortholog: 11q22.3 Cellular Component: mitochondrion; cytoplasm; cytosol Molecular Function:identical protein binding; protein homodimerization activity; metal ion binding; 6-pyruvoyltetrahydropterin synthase activity Biological Process: amino acid metabolic process; tetrahydrobiopterin biosynthetic process; central nervous system development; regulation of nitric-oxide synthase activity; nitric oxide metabolic process Disease: Hyperphenylalaninemia, Bh4-deficient, A |
| NCBI Summary: | The enzyme encoded by this gene catalyzes the elimination of inorganic triphosphate from dihydroneopterin triphosphate, which is the second and irreversible step in the biosynthesis of tetrahydrobiopterin from GTP. Tetrahydrobiopterin, also known as BH(4), is an essential cofactor and regulator of various enzyme activities, including enzymes involved in serotonin biosynthesis and NO synthase activity. Mutations in this gene result in hyperphenylalaninemia. [provided by RefSeq, Oct 2008] |
| UniProt Code: | Q03393 |
| NCBI GenInfo Identifier: | 417553 |
| NCBI Gene ID: | 5805 |
| NCBI Accession: | Q03393.1 |
| UniProt Secondary Accession: | Q03393,Q8WVG8, B0YJ87, |
| UniProt Related Accession: | Q03393 |
| Molecular Weight: | 145 |
| NCBI Full Name: | 6-pyruvoyl tetrahydrobiopterin synthase |
| NCBI Synonym Full Names: | 6-pyruvoyltetrahydropterin synthase |
| NCBI Official Symbol: | PTS |
| NCBI Official Synonym Symbols: | PTPS |
| NCBI Protein Information: | 6-pyruvoyl tetrahydrobiopterin synthase; PTP synthase |
| UniProt Protein Name: | 6-pyruvoyl tetrahydrobiopterin synthase |
| Protein Family: | PTS system EIIBC component |
| UniProt Gene Name: | PTS |
| UniProt Entry Name: | PTPS_HUMAN |
 | Western blot analysis of extracts of various cell lines, using PTS antibody (CAB6306) at 1:1000 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: 30s. |
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