• Target：
• PLOD1

• Fields：
• >>Lysine degradation;>>Metabolic pathways

• Gene Name：
• PLOD1 LLH PLOD

• Protein Name：
• PLOD1

• Human Gene Id：
• 5351

• Human Swiss Prot No：
• Q02809

• Mouse Gene Id：
• 18822

• Mouse Swiss Prot No：
• Q9R0E2

• Rat Swiss Prot No：
• Q63321

• Immunogen：
• Synthesized peptide derived from human PLOD1 AA range: 551-601

• Specificity：
• This antibody detects endogenous levels of PLOD1 at Human/Mouse/Rat

• Formulation：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• Source：
• Polyclonal, Rabbit,IgG

• Dilution：
• WB 1:500-2000;IHC 1:50-300; ELISA 2000-20000

• Purification：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• Concentration：
• 1 mg/ml

• Storage Stability：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• Molecular Weight(Da)：
• 80kD

• Background：
• 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：
• catalytic activity:Procollagen L-lysine + 2-oxoglutarate + O(2) = procollagen 5-hydroxy-L-lysine + succinate + CO(2).,cofactor:Ascorbate.,cofactor:Iron.,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.,disease:Defects in PLOD1 are the cause of Nevo syndrome [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.,function:Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in coll

• Subcellular Location：
• Rough endoplasmic reticulum membrane; Peripheral membrane protein; Lumenal side.

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