ATIC Antibody, FITC conjugated (PACO25118)
- SKU:
- PACO25118
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- FITC
Description
抗体名: | ATIC Antibody, FITC conjugated (PACO25118) |
抗体コード: | PACO25118 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Bifunctional purine biosynthesis protein PURH protein (301-592AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | FITC |
バックグラウンド: | Bifunctional enzyme that catalyzes 2 steps in purine biosynthesis. |
シノニム: | Bifunctional purine biosynthesis protein PURH [Cleaved into: Bifunctional purine biosynthesis protein PURH, N-terminally processed] [Includes: Phosphoribosylaminoimidazolecarboxamide formyltransferase (EC 2.1.2.3) (5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase) (AICAR transformylase); IMP cyclohydrolase (EC 3.5.4.10) (ATIC) (IMP synthase) (Inosinicase)], ATIC, PURH |
UniProt Protein Function: | ATIC: Bifunctional enzyme that catalyzes 2 steps in purine biosynthesis. Defects in ATIC are the cause of AICAR transformylase/IMP cyclohydrolase deficiency (AICAR). A neurologically devastating inborn error of purine biosynthesis. Patients excrete massive amounts of AICA-riboside in the urine and accumulate AICA- ribotide and its derivatives in erythrocytes and fibroblasts. AICAR causes profound mental retardation, epilepsy, dysmorphic features and congenital blindness. Belongs to the PurH family. |
UniProt Protein Details: | Protein type:Hydrolase; Nucleotide Metabolism - purine; Cofactor and Vitamin Metabolism - one carbon pool by folate; EC 3.5.4.10; Methyltransferase; EC 2.1.2.3 Chromosomal Location of Human Ortholog: 2q35 Cellular Component: mitochondrion; membrane; cytosol Molecular Function:protein homodimerization activity; IMP cyclohydrolase activity; phosphoribosylaminoimidazolecarboxamide formyltransferase activity Biological Process: purine ribonucleoside monophosphate biosynthetic process; tetrahydrofolate biosynthetic process; organ regeneration; response to inorganic substance; nucleobase, nucleoside and nucleotide metabolic process; nucleobase, nucleoside, nucleotide and nucleic acid metabolic process; dihydrofolate metabolic process; nucleoside metabolic process; cerebellum development; cerebral cortex development; purine base metabolic process; 'de novo' IMP biosynthetic process Disease: Aicar Transformylase/imp Cyclohydrolase Deficiency |
NCBI Summary: | This gene encodes a bifunctional protein that catalyzes the last two steps of the de novo purine biosynthetic pathway. The N-terminal domain has phosphoribosylaminoimidazolecarboxamide formyltransferase activity, and the C-terminal domain has IMP cyclohydrolase activity. A mutation in this gene results in AICA-ribosiduria. [provided by RefSeq, Sep 2009] |
UniProt Code: | P31939 |
NCBI GenInfo Identifier: | 23831360 |
NCBI Gene ID: | 471 |
NCBI Accession: | P31939.3 |
UniProt Secondary Accession: | P31939,Q13856, Q53S28, A8K202, E9PBU3, |
UniProt Related Accession: | P31939 |
Molecular Weight: | 592 |
NCBI Full Name: | Bifunctional purine biosynthesis protein PURH |
NCBI Synonym Full Names: | 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase |
NCBI Official Symbol: | ATIC |
NCBI Official Synonym Symbols: | PURH; AICAR; AICARFT; IMPCHASE; HEL-S-70p |
NCBI Protein Information: | bifunctional purine biosynthesis protein PURH; AICARFT/IMPCHASE; epididymis secretory sperm binding protein Li 70p; AICAR formyltransferase/IMP cyclohydrolase bifunctional enzyme; phosphoribosylaminoimidazolecarboxamide formyltransferase/IMP cyclohydrolase; 5-aminoimidazole-4-carboxamide-1-beta-D-ribonucleotide transformylase/inosinicase |
UniProt Protein Name: | Bifunctional purine biosynthesis protein PURH |
UniProt Synonym Protein Names: | 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase; AICAR transformylase |
UniProt Gene Name: | ATIC |
UniProt Entry Name: | PUR9_HUMAN |