MATN3 Antibody (PACO44150)
제품 코드를 사용하여 Assay Genie 메인 사이트를 통해 주문합니다.
주문- SKU:
- PACO44150
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | MATN3 Antibody (PACO44150) |
Antibody SKU: | PACO44150 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:200-1:1000, IHC:1:20-1:200 |
Species Reactivity: | Human, Mouse |
Immunogen: | Recombinant Human Matrilin-3 protein (277-486AA) |
Form: | Liquid |
Storage Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Purification Method: | Antigen Affinity Purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Western blot. All lanes: MATN3 antibody at 14µg/ml. Lane 1: NIH/3T3 whole cell lysate. Lane 2: Mouse thymus tissue. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 53, 49 kDa. Observed band size: 53 kDa.. | |
Immunohistochemistry of paraffin-embedded human adrenal gland tissue using PACO44150 at dilution of 1:100. | |
Immunohistochemistry of paraffin-embedded human kidney tissue using PACO44150 at dilution of 1:100. |
Background: | Major component of the extracellular matrix of cartilage and may play a role in the formation of extracellular filamentous networks. |
Synonyms: | Matrilin-3, MATN3 |
UniProt Protein Function: | 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. |
UniProt Protein Details: | 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 |
NCBI Summary: | 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] |
UniProt Code: | O15232 |
NCBI GenInfo Identifier: | 11321565 |
NCBI Gene ID: | 4148 |
NCBI Accession: | NP_002372.1 |
UniProt Secondary Accession: | O15232,Q4ZG02, |
UniProt Related Accession: | O15232 |
Molecular Weight: | 52,817 Da |
NCBI Full Name: | matrilin-3 |
NCBI Synonym Full Names: | matrilin 3 |
NCBI Official Symbol: | MATN3 |
NCBI Official Synonym Symbols: | HOA; OS2; EDM5; DIPOA; OADIP |
NCBI Protein Information: | matrilin-3; OTTHUMP00000200547 |
UniProt Protein Name: | Matrilin-3 |
Protein Family: | Matrilin |
UniProt Gene Name: | MATN3 |
UniProt Entry Name: | MATN3_HUMAN |