ATIC Antibody (PACO17377)
- SKU:
- PACO17377
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
抗体名: | ATIC Antibody (PACO17377) |
抗体コード: | PACO17377 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC |
推奨される希釈: | ELISA:1:1000-1:2000, WB:1:200-1:1000, IHC:1:25-1:100 |
反応性: | Human, Mouse, Rat |
免疫原: | Fusion protein of human ATIC |
憲法: | Liquid |
ストレージバッファ: | -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
精製方法: | Antigen affinity purification |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
Gel: 6%SDS-PAGE, Lysate: 40 μg, Lane: 231 cell, Primary antibody: PACO17377(ATIC Antibody) at dilution 1/350 dilution, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 20 seconds. | |
The image on the left is immunohistochemistry of paraffin-embedded Human brain tissue using PACO17377(ATIC Antibody) at dilution 1/25, on the right is treated with fusion protein. (Original magnification: x200). |
バックグラウンド: | 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. |
シノニム: | 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase/IMP cyclohydrolase |
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 |