MATN3, Polyclonal Antibody

MATN3; HOA; OS2; EDM5; DIPOA; OADIP; Matrilin-3; matrilin 3

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

MATN3 Antibody is affinity chromatography purified via peptide column.

Liquid

1 mg/mL (lot specific)

MATN3 antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Small volumes of anti-MATN3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MATN3 Antibody: Matrilins (MATNs) are a family of non-collagenous extra-cellular matrix (ECM) proteins consisting of four known members that have been proposed to play key roles in modulating cellular phenotypes during chondrogenesis of mesenchymal stem cells (MSCs). MATN1 and MATN3 are expressed specifically in cartilage and are among the most up-regulated ECM proteins during chondrogenesis. MATN3 is composed of a single N-terminal von Willebrand Factor A (vWFA) domain followed by four epidermal growth factor (EGF) repeats and a coiled-coil domain whereas MATN1 is composed of two vWFA domains separated by one EGF-like domain. MATN1 or MATN3 may play a role in modulating chondrogenesis during the chondrocyte differentiation process. Mutations of this gene have been associated with variety of inherited chondrodysplasias. Recent studies show that aberrant expression and processing of MATN3 are hallmarks of conventional cartilaginous neoplasms.

ELISA (EIA), Western Blot (WB), Immunocytochemistry (ICC)

MATN3 antibody can be used for detection of Matn3 by Western blot at 1 - 2 mug/mL. Antibody can also be used for immunocytochemistry starting at 5 mug/mL.

Western blot analysis of MATN3 in 3T3 cell lysate with MATN3 antibody at (A) 1 and (B) 2 μg/mL.

Immunocytochemistry of MATN3 in 3T3 cells with MATN3 antibody at 5 μg/mL.

48,317 Da

matrilin 3

matrilin-3

Matrilin-3

MATN3_HUMAN

This gene encodes a member of von Willebrand factor A domain containing protein family. This family of proteins is thought to be involved in the formation of filamentous networks in the extracellular matrices of various tissues. This protein contains two von Willebrand factor A domains; it is present in the cartilage extracellular matrix and has a role in the development and homeostasis of cartilage and bone. Mutations in this gene result in multiple epiphyseal dysplasia. [provided by RefSeq, Jul 2008]

MATN3: Major component of the extracellular matrix of cartilage and may play a role in the formation of extracellular filamentous networks. Defects in MATN3 are the cause of multiple epiphyseal dysplasia type 5 (EDM5). EDM 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. EDM is broadly categorized into the more severe Fairbank and the milder Ribbing types. EDM5 is relatively mild and clinically variable. It is primarily characterized by delayed and irregular ossification of the epiphyses and early-onset osteoarthritis. Defects in MATN3 are the cause of spondyloepimetaphyseal dysplasia MATN3-related (SEMD-MATN3). A bone disease characterized by disproportionate early-onset dwarfism, bowing of the lower limbs, lumbar lordosis and normal hands. Skeletal abnormalities include short, wide and stocky long bones with severe epiphyseal and metaphyseal changes, hypoplastic iliac bones and flat, ovoid vertebral bodies. Genetic variations in MATN3 are associated with susceptibility to osteoarthritis type 2 (OS2); also called osteoarthritis of distal interphalangeal joints (OADIP) or hand osteoarthritis (HOA). Osteoarthritis is a degenerative disease of the joints characterized by degradation of the hyaline articular cartilage and remodeling of the subchondral bone with sclerosis. Clinical symptoms include pain and joint stiffness often leading to significant disability and joint replacement. In the hand, osteoarthritis can develop in the distal interphalangeal and the first carpometacarpal (base of thumb) and proximal interphalangeal joints. Patients with osteoarthritis may have one, a few, or all of these sites affected.

Protein type: Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 2p24-p23

Cellular Component: proteinaceous extracellular matrix; extracellular region

Molecular Function: protein binding; extracellular matrix structural constituent

Biological Process: extracellular matrix organization and biogenesis; skeletal development

Disease: Osteoarthritis Susceptibility 2; Spondyloepimetaphyseal Dysplasia, Matrilin-3 Related; Epiphyseal Dysplasia, Multiple, 5

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