Home > Product > Antibody > Rabbit Anti-Collagen XI alpha 2 antibody
Collagen XI alpha 2; COBA2_HUMAN; COL11A2; Collagen alpha 2(XI); Collagen alpha-2(XI) chain; Collagen type XI alpha 2; DAQB-79P13.8; DFNA13; HGNC:2187; HKE5; PARP; STL3; COBA2_HUMAN.
Cat:
SL2924R
Species Reactivity:
Human,(predicted: Mouse,Rat,Dog,Pig,Horse,Rabbit,)
Immunogen:
KLH conjugated synthetic peptide derived from human Collagen XI alpha 2:1631-1736/1736
Format:
Liquid
Storage instructions:
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
WB=1:500-2000ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500IF=1:100-500(Paraffin sections need to do antigen repair)not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
Product Overview:
Tissue/cell: human esophageal cancer; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,SLC0005) at 37℃ for 20 min; Incubation: Anti-Collagen XI alpha 2 Polyclonal Antibody, Unconjugated(SL2924R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(SLC0010) staining
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Product PDFs
Datasheet:


May play an important role in fibrillogenesis by controlling lateral growth of collagen II fibrils.

Function:
May play an important role in fibrillogenesis by controlling lateral growth of collagen II fibrils.

Subunit:
Trimers composed of three different chains: alpha 1(XI), alpha 2(XI), and alpha 3(XI). Alpha 3(XI) is a post-translational modification of alpha 1(II). Alpha 1(V) can also be found instead of alpha 3(XI)=1(II).

Subcellular Location:
Secreted. extracellular space. extracellular matrix.

Post-translational modifications:
Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
A disulfide-bonded peptide called proline/arginine-rich protein or PARP is released from the N-terminus during extracellular processing and is subsequently retained in the cartilage matrix from which it can be isolated in significant amounts.

DISEASE:
Defects in COL11A2 are the cause of Stickler syndrome type 3 (STL3) [MIM:184168]. STL3 is an autosomal dominant non-ocular form of Stickler syndrome. Classical Stickler syndrome associates ocular signs with more or less complete forms of Pierre Robin sequence, bone disorders and sensorineural deafness. Ocular symptoms are absent in STL3. Robin sequence includes an opening in the roof of the mouth (a cleft palate), a large tongue (macroglossia), and a small lower jaw (micrognathia). Bones are affected by slight platyspondylisis and large, often defective epiphyses. Juvenile joint laxity is followed by early signs of arthrosis. The degree of hearing loss varies among affected individuals and may become more severe over time. Syndrome expressivity is variable.
Defects in COL11A2 are the cause of autosomal recessive otospondylomegaepiphyseal dysplasia (OSMED) [MIM:215150]. OSMED is a skeletal dysplasia accompanied by severe hearing loss. The phenotype overlaps that of autosomal dominant skeletal disorders (Stickler and Marshall syndromes) but can be distinguished by disproportionately short limbs and lack of ocular involvement.
Defects in COL11A2 are the cause of Weissenbacher-Zweymueller syndrome (WZS) [MIM:277610]. WZS is an autosomal dominant disorder allelic with STL3 and OSMED. WZS is also referred to as heterozygous OSMED.
Defects in COL11A2 are the cause of deafness autosomal dominant type 13 (DFNA13) [MIM:601868]. DFNA13 is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information.
Defects in COL11A2 are the cause of deafness autosomal recessive type 53 (DFNB53) [MIM:609706].

Similarity:
Belongs to the fibrillar collagen family.
Contains 1 fibrillar collagen NC1 domain.
Contains 1 TSP N-terminal (TSPN) domain.

SWISS:
P13942

Gene ID:
1302

Database links:

Entrez Gene: 1302 Human

Omim: 120290 Human

SwissProt: P13942 Human

SwissProt: Q5JP94 Human

SwissProt: Q5STP6 Human

SwissProt: Q5SUI8 Human

SwissProt: Q7Z6C3 Human

Unigene: 390171 Human



Picture

Tissue/cell: human esophageal cancer; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,SLC0005) at 37℃ for 20 min;
Incubation: Anti-Collagen XI alpha 2 Polyclonal Antibody, Unconjugated(SL2924R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(SLC0010) staining
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