製品名Native Human Collagen VI protein
Collagen VI タンパク質・ペプチド 製品一覧
タンパク質長Full length protein
配列の追加情報Prepared from Human Placenta.
Our Abpromise guarantee covers the use of ab7538 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
This product is free from other collagens, human serum proteins and non-collagen extracellular matrix proteins. This product reacts with anti-Collagen Type VI. Reaction with anti-Collagen I, II, III, IV or V is negligible (typically less than 1% cross reactivity was detected by ELISA).
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Shipped at 4°C. Store at +4°C. Diluted stock solutions should be used immediately and not be frozen. Please see notes section. Store undiluted.
Preservative: 0.01% Sodium azide
Constituent: 3% Acetic acid
- Alpha 1 (VI) chain (61 AA)
機能Collagen VI acts as a cell-binding protein.
関連疾患Defects in COL6A1 are a cause of Bethlem myopathy (BM) [MIM:158810]. BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles.
Defects in COL6A1 are a cause of Ullrich congenital muscular dystrophy (UCMD) [MIM:254090]; also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy.
配列類似性Belongs to the type VI collagen family.
Contains 3 VWFA domains.
翻訳後修飾Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
細胞内局在Secreted > extracellular space > extracellular matrix.
- Information by UniProt
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab7538 は 2 報の論文で使用されています。
- Holm Nielsen S et al. A Fragment of Collagen Type VI alpha-3 chain is Elevated in Serum from Patients with Gastrointestinal Disorders. Sci Rep 10:5910 (2020). PubMed: 32245981
- Veidal SS et al. MMP Mediated Degradation of Type VI Collagen Is Highly Associated with Liver Fibrosis - Identification and Validation of a Novel Biochemical Marker Assay. PLoS One 6:e24753 (2011). PubMed: 21935455