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Catalogue Number : ECM1040N
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ArthroMAB; Kit, ten mice

Recommended Replacement for: ECM1040

Species Reactivity	Key Applications	Host	Format	Antibody Type
M	ACT, FUNC	Mouse	null	Monoclonal Antibody

UniProt Number:

P02458

UniProt Summary

FUNCTION: SwissProt: P02458 # Type II collagen is specific for cartilaginous tissues. It is essential for the normal embryonic development of the skeleton, for linear growth and for the ability of cartilage to resist compressive forces.
SIZE: 1487 amino acids; 141785 Da
SUBUNIT: Homotrimers of alpha 1(II) chains.
SUBCELLULAR LOCATION: Secreted, extracellular space, extracellular matrix (By similarity).
TISSUE SPECIFICITY: High expression of isoform 2 in juvenile chondrocyte and low in fetal chondrocyte.
PTM: Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains. & The N-telopeptide is covalently linked to the helical COL2 region of alpha 1(IX), alpha 2(IX) and alpha 3(IX) chain. The C- telopeptide is covalently linked to an another site in the helical region of alpha 3(IX) COL2.
DISEASE: SwissProt: P02458 # Defects in COL2A1 are the cause of a variety of chondrodysplasia including hypochondrogenesis and osteoarthritis. & Defects in COL2A1 are the cause of spondyloepiphyseal dysplasia congenital type (SEDC) [MIM:183900]. This disorder is characterized by disproportionate short stature and pleiotropic involvement of the skeletal and ocular systems. & Defects in COL2A1 are the cause of Strudwick type spondyloepimetaphyseal dysplasia (SEMD) [MIM:184250]. SEMD is characterized by disproportionate short stature, pectus carinatum, and scoliosis, as well as dappled metaphyses (which is not seen in SEDC). & Defects in COL2A1 are the cause of achondrogenesis hypochondrogenesis type 2 (ACG2) [MIM:200610]. ACG2 is a disease characterized by the absence of ossification in the vertebral column, sacrum and pubic bones. & Defects in COL2A1 are the cause of Legg-Calve-Perthes disease (LCPD) [MIM:150600]; also known as Legg-Perthes disease or Perthes disease. LCPD is characterized by loss of circulation to the femoral head, resulting in avascular necrosis in a growing child. Clinical pictures of the disease vary, depending on the phase of disease progression through ischemia, revascularization, fracture and collapse, and repair and remodeling of the bone. & Defects in COL2A1 are the cause of Kniest syndrome (KS) [MIM:156550]; also known as Kniest dysplasia or metatropic dwarfism type II. KS is a moderately severe chondrodysplasia phenotype that results from mutations in the COL2A1 gene. Characteristics of the disorder include a short trunk and extremities, mid-face hypoplasia, cleft palate, myopia, retinal detachment, and hearing loss. & Defects in COL2A1 are a cause of primary avascular necrosis of femoral head (ANFH) [MIM:608805]; also called ischemic necrosis of the femoral head or osteonecrosis of the femoral head. ANFH causes disability that often requires surgical intervention. Most cases are sporadic, but families in which there is an autosomal dominant inheritance of the disease have been identified. It has been estimated that 300,000 to 600,000 people in the United States have ANFH. Approximately 15,000 new cases of this common and disabling disorder are reported annually. The age at the onset is earlier than that for osteoarthritis. The diagnosis is typically made when patients are between the ages of 30 and 60 years. The clinical manifestations, such as pain on exertion, a limping gait, and a discrepancy in leg length, cause considerable disability. Moreover, nearly 10 percent of the 500,000 total-hip arthroplasties performed each year in the United States involve patients with ANFH. As a result, this disease creates a substantial socioeconomic cost as well as a burden for patients and their families. & Defects in COL2A1 are the cause of osteoarthritis with mild chondrodysplasia [MIM:604864]. Osteoarthritis is a common disease that produces joint pain and stiffness together with radiologic evidence of progressive degeneration of joint cartilage. Some forms of osteoarthritis are secondary to events such as trauma, infections, metabolic disorders, or congenital or heritable conditions that deform the epiphyses or related structures. In most patients, however, there is no readily identifiable cause of osteoarthritis. Inheritance in a Mendelian dominant manner has been demonstrated in some families with primary generalized osteoarthritis. Reports demonstrate coinheritance of primary generalized osteoarthritis with specific alleles of the gene COL2A1, the precursor of the major protein of cartilage. & Defects in COL2A1 are the cause of platyspondylic lethal skeletal dysplasia Torrance type (PLSD-T) [MIM:151210]. Platyspondylic lethal skeletal dysplasias (PLSDs) are a heterogeneous group of chondrodysplasias characterized by severe platyspondyly and limb shortening. PLSD-T is characterized by varying platyspondyly, short ribs with anterior cupping, hypoplasia of the lower ilia with broad ischial and pubic bones, and shortening of the tubular bones with splayed and cupped metaphyses. Histology of the growth plate typically shows focal hypercellularity with slightly enlarged chondrocytes in the resting cartilage and relatively well-preserved columnar formation and ossification at the chondro-osseous junction. PLSD-T is generally a perinatally lethal disease, but a few long-term survivors have been reported. & Defects in COL2A1 are the cause of multiple epiphyseal dysplasia with myopia and conductive deafness (EDMMD) [MIM:132450]. Multiple epiphyseal dysplasia is a generalized skeletal dysplasia associated with significant morbidity. Joint pain, joint deformity, waddling gait, and short stature are the main clinical signs and symptoms. EDMMD is an autosomal dominant disorder characterized by epiphyseal dysplasia associated with progressive myopia, retinal thinning, crenated cataracts, conductive deafness. & Defects in COL2A1 are the cause of spondyloperipheral dysplasia (SPD) [MIM:271700]. SPD patients manifest short stature, midface hypoplasia, sensorineural hearing loss, spondyloepiphyseal dysplasia, platyspondyly and brachydactyly. & Defects in COL2A1 are the cause of Wagner syndrome type II (WS-II); a disease characterized by early-onset cataracts, lattice degeneration of the retina, and retinal detachment without involvement of monocular tissues. & Defects in COL2A1 are the cause of Stickler syndrome type 1 (STL1) [MIM:108300]; also known as vitreous type 1, or membranous vitreous type. STL1 is an autosomal dominant disorder characterized by progressive myopia beginning in the first decade of life, vitreo-retinal degeneration, retinal detachment, cleft palate, midfacial hypoplasia, osteoarthritis, and sensorineural hearing loss. & Defects in COL2A1 are a cause of autosomal dominant rhegmatogenous retinal detachment (DRRD) [MIM:609508]. RDD most frequently results from retinal tearing at the time of posterior vitreous detachment. Non-syndromic RRD can be inherited in a clearly dominant fashion, although in most of these cases, the genetic locus for the disorder is unknown. However, RRD is also a common feature of the type II collagenopathies (disorders due to mutations in the gene COL2A1) and some recent examples of mutations in this gene suggest that COL2A1 should be considered a candidate gene for dominant RRD (DRRD). & Of special interest are three different variants that replace arginine codons at positions 275, 719 and 989 in the triple-helical domain with codons for cysteine, an amino acid not normally found in the triple-helical domain of type II collagen from any species. They are of special interest, because they are the only amino acid substitutions in the triple-helical domain that replaces a Y-position amino acid and cause a disease phenotype. Also, they are recurrent in that they have been found in more than one unrelated individual.
SIMILARITY: SwissProt: P02458 ## Belongs to the fibrillar collagen family. & Contains 1 VWFC domain. ... see more »

Entrez Gene Number:

NM_001844.4

Entrez Gene Summary

This gene encodes the alpha-1 chain of type II collagen, a fibrillar collagen found in cartilage and the vitreous humor of the eye. Mutations in this gene are associated with achondrogenesis, chondrodysplasia, early onset familial osteoarthritis, SED congenita, Langer-Saldino achondrogenesis, Kniest dysplasia, Stickler syndrome type I, and spondyloepimetaphyseal dysplasia Strudwick type. In addition, defects in processing chondrocalcin, a calcium binding protein that is the C-propeptide of this ... see more »

Description:

ArthroMAB; Kit, ten mice

Replaces:

ECM1040

Key Applications:

Activity Assay
Affects Function

Species Reactivity:

Mouse

Presentation:

The antibody cocktail contains 10 mM Sodium Phosphate, 125 mM NACL, pH 7.1 with no preservatives.

Storage Conditions:

Materials should be stored at -20°C for up to one year from date of receipt. Avoid repeated freeze/thaw of monoclonal antibodies.

Precautions:
LPS is pyrogenic. This material should be handled by trained personnel with appropriate laboratory precautions.

Gene Symbol:

COL2A1
SEDC
COL11A3
ANFH
MGC131516
chondrocalcin
AOM

Components:

ArthroMAB Monoclonal Antibody Blend - (Catalog No. 1008547) 4.0 mL of monoclonal antibody cocktail solution containing 10 mg each of four monoclonal antibodies (clones C11b, J1, D3 and U1) in sterile PBS. Total protein concentration = 10.0 mg/mL.
LPS - (Catalog No. 90032) 2 vials containing 500 ug Lipopolysaccharide from E. coli strain 0111B4 dissolved in PBS at 1mg/mL

Antibody Category:

Inflammation & Immunology

Antibody Sub-Category:

Inflammation & Autoimmune Mechanisms

Usage Statement:

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
This product is manufactured for Millipore by MD Biosciences, Morwell Diagnostics, Switzerland.

Descriptive Text:

Trade Name:

Chemicon (Millipore)

Packaging:

40 mg

Product Overview:

Collagen-induced arthritis (CIA) in mice is widely used as an experimental model for rheumatoid arthritis (RA) in humans. CIA is mediated by autoantibodies, which bind to a particular region of type II collagen, such as CB11 (CNBR-digested fragment of type II collagen) (Terato et al, 1985). Antibody-mediated CIA can be induced by i.v. injection of a combination of four different monoclonal antibodies generated by the ArthroMAB mouse B-hybridoma cell lines. T In the mouse CIA model, the antibody response that is correlated with arthritis is mainly associated with binding to the epitopes of C11b, J1 and U1. This cocktail of 4 purified mouse monoclonal antibodies bind to the well-defined epitopes C11b, J1, D3 and U1, which are spread over the entire CII (CB8, CB10 and CB11 fragments), possibly encouraging better immune complex formation on the cartilage surface for the initiation of arthritis (Terato et al, 1985).

Antibody Type:

Monoclonal Antibody

Host:

Mouse