ADAMTSL4 Antibody

說明書

中文名稱：

ADAMTSL4兔多克隆抗體
貨號：

CSB-PA761506ESR1HU
規格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA761506ESR1HU at dilution of 1:100

Immunohistochemistry of paraffin-embedded human adrenal gland tissue using CSB-PA761506ESR1HU at dilution of 1:100
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) ADAMTSL4 Polyclonal antibody
Uniprot No.：

Q6UY14
基因名：

ADAMTSL4
別名：

ADAMTSL4 antibody; TSRC1 antibody; PP1396 antibody; UNQ2803/PRO34012ADAMTS-like protein 4 antibody; ADAMTSL-4 antibody; Thrombospondin repeat-containing protein 1 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human ADAMTS-like protein 4 protein (795-1074AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產品提供形式：

Liquid
應用范圍：

ELISA, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

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

產品評價

靶點詳情

功能：

Positive regulation of apoptosis. May facilitate FBN1 microfibril biogenesis.
基因功能參考文獻：
1. A recurrent pathogenic ADAMTSL4 variant is a major cause of early onset autosomal recessive ectopia lentis in a Cook Island Maori population and associated with a common haplotype, suggesting a founder effect. PMID: 28394649
2. study reports a novel founder mutation in ADAMTSL4 gene in children of Bukharian Jewish origin presenting with early-onset bilateral ectopia lentis PMID: 26653794
3. ADAMTSL4 mutations are the main cause for isolated ectopia lentis with an early onset of symptoms and possible severe ocular complications. PMID: 25975359
4. Compound heterozygous c.1783dupT and c. 2594G>A mutations of ADAMTSL4 gene were causative mutations for this family with isolated non-syndromic ectopia lentis PMID: 24802351
5. We have confirmed the gene and protein expression of ADAMTSL4 in human ocular tissue. The pattern of expression may suggest further functions of this gene beyond those suggested by its causative role in isolated ectopia lentis. PMID: 23846871
6. This is the first detailed report of a possible genetic determinant of craniosynostosis with ectopia lentis. PMID: 22871183
7. Patients from a family with ectopia lentis et pupillae (ELP) in four generations have autosomal recessive ELP caused by novel mutations in ADAMTSL4. PMID: 23426735
8. Mutations in ADAMTSL4 appear to cause earlier manifestation of ectopia lentis and are associated with increased axial length. PMID: 22736615
9. Enhanced fibrillin-1 deposition in the presence of ADAMTSL4 and colocalization of ADAMTSL4 with fibrillin-1 in the ECM of cultured fibroblasts suggest a potential role for ADAMTSL4 in the formation or maintenance of the zonule. PMID: 21989719
10. The results emphasize the association of ADAMTSL4 null mutations with isolated ectopia lentis and the presence of a founder mutation in the European population. PMID: 21051722
11. Ectopia lentis et pupillae is associated with a number of malformations primarily in the anterior segment of the eye. PMID: 20702823
12. This study confirms that homozygous mutations in ADAMTSL4 are associated with autosomal-recessive ectopia lentis in British families. PMID: 20564469
13. Herein we show a consanguineous family that carries a novel homozygous splice mutation IVS4-1G>A/IVS4-1G>A in ADAMTSL4 responsible for isolated autosomal recessive ectopia lentis. PMID: 20141359
14. Mutations in ADAMTSL4 are responsible for autosomal-recessive simple ectopia lentis and this proteins affectsthe development of the zonular fibers. PMID: 19200529
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相關疾病：

Ectopia lentis 2, isolated, autosomal recessive (ECTOL2); Ectopia lentis et pupillae (ECTOLP)
亞細胞定位：

Secreted, extracellular space, extracellular matrix. Note=Colocalizes with FMN1 microfibrils in the eye ECM.
組織特異性：

Expressed in colon, heart, leukocyte, liver, lung, skeletal muscle, spleen, testis and placenta. Weaker expression in bone marrow, brain tissue, kidney and pancreas. Expression studies in fetal tissues reveal strong expression in heart, kidney, liver, lun
數據庫鏈接：

HGNC: 19706

OMIM: 225100

KEGG: hsa:54507

STRING: 9606.ENSP00000271643

UniGene: Hs.516243

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