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Matrilin 3 Rabbit pAb (bs-18694R)  
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50ul/1180.00元
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產(chǎn)品編號 bs-18694R
英文名稱 Matrilin 3 Rabbit pAb
中文名稱 胞外基質(zhì)蛋白3抗體
別    名 AV009181; DIPOA; EDM5; HOA; MATN3; MATN3_HUMAN; Matrilin 3; Matrilin-3; OADIP; OS2.  
Specific References  (1)     |     bs-18694R has been referenced in 1 publications.
[IF=5.076] Guo Zhu. et al. Exosomal MATN3 of Urine-Derived Stem Cells Ameliorates Intervertebral Disc Degeneration by Antisenescence Effects and Promotes NPC Proliferation and ECM Synthesis by Activating TGF-β. Oxid Med Cell Longev. 2021;2021:5542241  WB,IF ;  Rat.  
研究領(lǐng)域 細胞生物  發(fā)育生物學  信號轉(zhuǎn)導  細胞外基質(zhì)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human,Mouse,Rat,Rabbit,Pig,Cow,Dog,Cat,GuineaPig,Horse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 53 kDa
檢測分子量
細胞定位 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Matrilin 3: 51-150/486 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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]

Function:
Major component of the extracellular matrix of cartilage and may play a role in the formation of extracellular filamentous networks.

Subcellular Location:
Secreted.

Tissue Specificity:
Expressed only in cartilaginous tissues, such as vertebrae, ribs and shoulders.

DISEASE:
Defects in MATN3 are the cause of multiple epiphyseal dysplasia type 5 (EDM5) [MIM:607078]. 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) [MIM:608728]. 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) [MIM:140600]; 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.

Similarity:
Contains 4 EGF-like domains.
Contains 1 VWFA domain.

SWISS:
O15232

Gene ID:
4148

Database links:

Entrez Gene: 4148 Human

Entrez Gene: 17182 Mouse

Entrez Gene: 313954 Rat

Omim: 602109 Human

SwissProt: O15232 Human

SwissProt: O35701 Mouse

Unigene: 656199 Human

Unigene: 42226 Mouse



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