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Conditions of optimal Collagen III α1 antibody performance should be determined experimentally by the investigator.
Price/availability/specifications subject to change without notice. Unless otherwise indicated, our catalog and customized products are for research use only and not intended for human or animal diagnostic or therapeutic use.
Phone: 1-617-401-8149
Fax: 1-617-606-5019
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Or leave a message with a formal purchase
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Introduction
Type III collagen is a fibrillar forming collagen comprising three α1(III) chains and is expressed in early embryos and throughout embryogenesis. In the adult, type III collagen is a major component of the extracellular matrix in a variety of internal organs and skin. Mutations in the COL3A1 gene have been implicated as a cause of type IV Ehlers?Danlos syndrome, a disease leading to aortic rupture in early adult life. To directly study the role of Col3a1 in development and disease, we have inactivated the Col3a1 gene in embryonic stem cells by homologous recombination. The mutated allele was transmitted through the mouse germ line and homozygous mutant animals were derived from heterozygous intercrosses. About 10% of the homozygous mutant animals survived to adulthood but have a much shorter life span compared with wild-type mice. The major cause of death of mutant mice was rupture of the major blood vessels, similar to patients with type IV Ehlers?Danlos syndrome. Ultrastructural analysis of tissues from mutant mice revealed that type III collagen is essential for normal collagen I fibrillogenesis in the cardiovascular system and other organs.
PA001263-L0211: Collagen III α1 Polyclonal Antibody
Rabbit Polyclonal Antibody.
Specificity/Sensitivity: Collagen III α1 (Cleaved-Gly1221) antibody detects endogenous levels of fragment of activated Collagen III α1 resulting from cleavage adjacent to Gly1221.
Applications: WB: 1:500~1:1000 ELISA: 1:10000.
Reactivity: Human.
Immunogen: The antiserum was produced against synthesized peptide derived from human Collagen III α1.
Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Form of Antibody: Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150 mM NaCl, 0.02% sodium azide and 50% glycerol.
Storage/Stability: Stable for 1 year at -20°C and 3 months at 4°C. For maximum recovery of the product, centrifuge the original vial after thawing and before removing the cap. Aliquot to avoid repeated freezing and thawing.
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