Mouse Anti-Collagen III antibody

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COL 3A1; COL3A1; Collagen alpha 1(III) chain; Collagen III alpha 1 chain precursor; Collagen III alpha 1 polypeptide; Collagen type III alpha 1 (Ehlers Danlos syndrome type IV autosomal dominant); Collagen type III alpha 1; Collagen type III alpha; EDS4A;

Cat:

SLM33129M

Species Reactivity：

Human,(predicted: Mouse,Rat,)

Immunogen：

Recombinant human Collagen III protein

Format：

Liquid

Storage instructions：

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Concentration：

1mg/ml

Clonality：

Monoclonal

Isotype：

IgG

Applications：

WB=1:500-1000not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Mouse

Product Overview：

Sample: A549 Cell (Human) Lysate at 40 ugPrimary: Anti-Collagen III (SLM33129M) at 1/1000 dilutionSecondary: IRDye800CW Goat Anti-Mouse IgG at 1/20000 dilutionPredicted band size: 117 kDObserved band size: 117 kD

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Unit:

Price: $228.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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The This gene encodes the pro-alpha1 chains of type III collagen, a fibrillar collagen that is found in extensible connective tissues such as skin, lung, uterus, intestine and the vascular system, frequently in association with type I collagen. Mutations in this gene are associated with Ehlers-Danlos syndrome types IV, and with aortic and arterial aneurysms. Two transcripts, resulting from the use of alternate polyadenylation signals, have been identified for this gene. [provided by R. Dalgleish, Feb 2008]

Function:
Collagen type III occurs in most soft connective tissues along with type I collagen.

Subunit:
Trimers of identical alpha 1(III) chains. The chains are linked to each other by interchain disulfide bonds. Trimers are also cross-linked via hydroxylysines.

Subcellular Location:
Secreted, extracellular space, extracellular matrix.

Post-translational modifications:
Proline residues at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
O-linked glycan consists of a Glc-Gal disaccharide bound to the oxygen atom of a post-translationally added hydroxyl group.

DISEASE:
Defects in COL3A1 are a cause of Ehlers-Danlos syndrome type 3 (EDS3) [MIM:130020]; also known as benign hypermobility syndrome. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS3 is a form of Ehlers-Danlos syndrome characterized by marked joint hyperextensibility without skeletal deformity.
Defects in COL3A1 are the cause of Ehlers-Danlos syndrome type 4 (EDS4) [MIM:130050]. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS4 is the most severe form of the disease. It is characterized by the joint and dermal manifestations as in other forms of the syndrome, characteristic facial features (acrogeria) in most patients, and by proneness to spontaneous rupture of bowel and large arteries. The vascular complications may affect all anatomical areas.
Defects in COL3A1 are a cause of susceptibility to aortic aneurysm abdominal (AAA) [MIM:100070]. AAA is a common multifactorial disorder characterized by permanent dilation of the abdominal aorta, usually due to degenerative changes in the aortic wall. Histologically, AAA is characterized by signs of chronic inflammation, destructive remodeling of the extracellular matrix, and depletion of vascular smooth muscle cells.

Similarity:
Belongs to the fibrillar collagen family.
Contains 1 fibrillar collagen NC1 domain.
Contains 1 VWFC domain.

SWISS:
P02461

Gene ID:
1281

Database links:

Entrez Gene: 1281 Human

Entrez Gene: 12825 Mouse

Entrez Gene: 16832 Rat

Omim: 12036 Human

SwissProt: P02461 Human

SwissProt: P08121 Mouse

SwissProt: P13941 Rat

Unigene: 443625 Human

Unigene: 249555 Mouse

Unigene: 3247 Rat

Picture

Sample: A549 Cell (Human) Lysate at 40 ug
Primary: Anti-Collagen III (SLM33129M) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Mouse IgG at 1/20000 dilution
Predicted band size: 117 kD
Observed band size: 117 kD

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