產(chǎn)品編號(hào) | bsm-51740M |
英文名稱 | Mouse Anti-PLOD1 antibody |
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(lǐng)域 | 染色質(zhì)和核信號(hào) 轉(zhuǎn)錄調(diào)節(jié)因子 表觀遺傳學(xué) |
抗體來(lái)源 | Mouse |
克隆類型 | Monoclonal |
克 隆 號(hào) | M4G1 |
交叉反應(yīng) | Human |
產(chǎn)品應(yīng)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 | 33kDa |
細(xì)胞定位 | 細(xì)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,κ |
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(xiàng) | 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 |
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 |
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