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PLOD1 Mouse mAb (bsm-51740M)  
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產品編號 bsm-51740M
英文名稱 PLOD1 Mouse mAb
中文名稱 賴氨酸羥化酶1單克隆抗體
別    名 PLOD; PLOD1_HUMAN; 2-oxoglutarate 5-dioxygenase 1; EDS6; LH; LH1; LLH; Lysine hydroxylase; Lysyl hydroxylase 1; Procollagen lysine 1 2 oxoglutarate 5 dioxygenase 1; Procollagen lysine 2 oxoglutarate 5 dioxygenase 1; Procollagen-lysine.  
研究領域 染色質和核信號  轉錄調節(jié)因子  表觀遺傳學  
抗體來源 Mouse
克隆類型 Monoclonal
克 隆 號 M4G1
交叉反應 Human
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 33 kDa
檢測分子量
細胞定位 細胞核 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PLOD1: 51-150/727 
亞    型 IgG2b,k
純化方法 affinity purified by Protein G
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
產品介紹 Lysyl hydroxylase is a membrane-bound homodimeric protein localized to the cisternae of the endoplasmic reticulum. The enzyme (cofactors iron and ascorbate) catalyzes the hydroxylation of lysyl residues in collagen-like peptides. The resultant hydroxylysyl groups are attachment sites for carbohydrates in collagen and thus are critical for the stability of intermolecular crosslinks. Some patients with Ehlers-Danlos syndrome type VI have deficiencies in lysyl hydroxylase activity. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2015]

Function:
Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links.

Subcellular Location:
Rough endoplasmic reticulum membrane.

DISEASE:
Defects in PLOD1 are the cause of Ehlers-Danlos syndrome type 6 (EDS6) [MIM:225400]. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS6 is characterized by the presence of ocular complications, particularly retinal detachment.
Defects in PLOD1 are the cause of Nevo syndrome (NEVOS) [MIM:601451]. This is a rare, autosomal recessive disorder characterized by increased perinatal length, kyphosis, muscular hypotonia, and joint laxity. Nevo syndrome and EDS-VI have similar clinical phenotypes. Some authors consider that both syndromes are the same clinical entity.

Similarity:
Contains 1 Fe2OG dioxygenase domain.

SWISS:
Q02809

Gene ID:
5351

Database links:

Entrez Gene: 5351 Human

Omim: 153454 Human

SwissProt: Q02809 Human

Unigene: 75093 Human

產品圖片
Sample: Lane 1: U-87MG cell lysates Lane 2: K562 cell lysates Lane 3: HeLa cell lysates Lane 4: A431 cell lysates Primary: Anti-PLOD1 (bsm-51740M) at 1/4000 dilution Secondary: IRDye800CW Goat Anti-Mouse IgG at 1/20000 dilution Predicted band size: 33 kD Observed band size: 85 kD
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