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Rabbit Anti-PLOD3/AF555 Conjugated antibody
Rabbit Anti-PLOD3/AF555 Conjugated antibody
2-oxoglutarate 5-dioxygenase 3; bone protein I; BP-I; LH3; Lysine hydroxylase 3; Lysyl hydroxylase 3; Plod3; PLOD3_HUMAN; Procollagen lysine,2 oxoglutarate 5 dioxygenase 3 precursor; Procollagen-lysine.
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  • NO.:SL12732R-AF555
    Clonality:Polyclonal
    Immunogen Species:Rabbit
    React Species:Mouse,(predicted: Human,Rat,Dog,Pig,Cow,Horse,Rabbit,Sheep,)
    Applications:ICC IF
    concentration:1mg/ml
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Details

Product Name Anti-PLOD3/AF555
Chinese Name AF555标记的赖氨酸羟化酶3抗体
Alias 2-oxoglutarate 5-dioxygenase 3; bone protein I; BP-I; LH3; Lysine hydroxylase 3; Lysyl hydroxylase 3; Plod3; PLOD3_HUMAN; Procollagen lysine,2 oxoglutarate 5 dioxygenase 3 precursor; Procollagen-lysine.  
Research Area Cell biology  immunology  The cell membrane蛋白  
Immunogen Species Rabbit
Clonality Polyclonal
React Species Mouse,  (predicted: Human, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
Applications ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
Molecular weight 82kDa
Form Lyophilized or Liquid
Concentration 1mg/ml
immunogen KLH conjugated synthetic peptide derived from human PLOD3
Lsotype IgG
Purification affinity purified by Protein A
Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
Storage Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail background:
The protein encoded by this gene is a membrane-bound homodimeric enzyme that is localized to the cisternae of the rough 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 VIB have deficiencies in lysyl hydroxylase activity. [provided by RefSeq, Jul 2008]

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 PLOD3 are the cause of lysyl hydroxylase 3 deficiency (LH3 deficiency) [MIM:612394]; also known as bone fragility with contractures arterial rupture and deafness. LH3 deficiency is a connective tissue disorder. The syndrome is characterized by congenital malformations severely affecting many tissues and organs and revealing features of several collagen disorders, most of them involving COL2A1 (type II collagen). The findings suggest that the failure of lysyl hydroxylation and hydroxylysyl carbohydrate addition, which affects many collagens, is the molecular basis of this syndrome.

Similarity:
Contains 1 Fe2OG dioxygenase domain.

Database links:

Entrez Gene: 8985 Human

Entrez Gene: 26433 Mouse

Entrez Gene: 288583 Rat

Omim: 603066 Human

SwissProt: O60568 Human

SwissProt: Q9R0E1 Mouse

SwissProt: Q5U367 Rat

Unigene: 153357 Human

Unigene: 251003 Mouse

Unigene: 90152 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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