Description
| 抗体名: | CRTAP Antibody (PACO08610) |
| 抗体コード: | PACO08610 |
| サイズ: | 50ul |
| 宿主種: | Rabbit |
| 申し込み: | ELISA, WB, IHC |
| 推奨される希釈: | |
| 反応性: | Human, Mouse, Rat |
| 免疫原: | Human CRTAP |
| 憲法: | Liquid |
| ストレージバッファ: | PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
| 精製方法: | Antigen Affinity purified |
| 抗体のクローン性: | Polyclonal |
| アイソタイプ: | IgG |
| Conjugate: | Non-conjugated |
| シノニム: | cartilage associated protein;CRTAP;CASP;LEPREL3 ; |
| UniProt Protein Function: | CRTAP: Necessary for efficient 3-hydroxylation of fibrillar collagen prolyl residues. Defects in CRTAP are the cause of osteogenesis imperfecta type 7 (OI7). A connective tissue disorder characterized by short stature, short humeri and femora, coxa vara, white sclera, and the absence of dentinogenesis imperfecta. Multiple fractures are present at birth, and patients manifest moderate-severe bone fragility. Death may occurr in the perinatal period due to secondary respiratory insufficiency. Belongs to the leprecan family. |
| UniProt Protein Details: | Protein type:Extracellular matrix; Secreted, signal peptide; Secreted Chromosomal Location of Human Ortholog: 3p22.3 Cellular Component: proteinaceous extracellular matrix; extracellular space; endoplasmic reticulum lumen; endoplasmic reticulum Molecular Function:protein complex binding Biological Process: extracellular matrix organization and biogenesis; protein stabilization; spermatogenesis; peptidyl-proline hydroxylation to 3-hydroxy-L-proline Disease: Osteogenesis Imperfecta, Type Vii |
| NCBI Summary: | The protein encoded by this gene is similar to the chicken and mouse CRTAP genes. The encoded protein is a scaffolding protein that may influence the activity of at least one member of the cytohesin/ARNO family in response to specific cellular stimuli. Defects in this gene are associated with osteogenesis imperfecta, a connective tissue disorder characterized by bone fragility and low bone mass. [provided by RefSeq, Jul 2008] |
| UniProt Code: | O75718 |
| NCBI GenInfo Identifier: | 17372894 |
| NCBI Gene ID: | 10491 |
| NCBI Accession: | O75718.1 |
| UniProt Related Accession: | O75718 |
| Molecular Weight: | 46562 |
| NCBI Full Name: | Cartilage-associated protein |
| NCBI Synonym Full Names: | cartilage associated protein |
| NCBI Official Symbol: | CRTAP |
| NCBI Official Synonym Symbols: | OI7; CASP; P3H5; LEPREL3 |
| NCBI Protein Information: | cartilage-associated protein |
| UniProt Protein Name: | Cartilage-associated protein |
| Protein Family: | Cartilage-associated protein |
| UniProt Gene Name: | CRTAP |
| UniProt Entry Name: | CRTAP_HUMAN |
