product summary
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company name :
MyBioSource
product type :
antibody
product name :
PLOD3 Polyclonal Antibody
catalog :
MBS126263
quantity :
0.1 mL
price :
275 USD
clonality :
polyclonal
host :
unidentified
conjugate :
nonconjugated
reactivity :
human
application :
western blot
more info or order :
image
image 1 :
Western blot analysis of extracts of various cell lines, using PLOD3 antibody. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST.
product information
catalog number :
MBS126263
products type :
Antibody
products full name :
PLOD3 Polyclonal Antibody
products short name :
[PLOD3]
products name syn :
[LH3]
other names :
[Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3; Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3; procollagen-lysine,2-oxoglutarate 5-dioxygenase 3; lysyl hydroxlase 3; lysyl hydroxylase 3; lysine hydroxylase 3; procollagen-lysine, 2-oxoglutarate 5-dioxygenase 3; Lysyl hydroxylase 3; LH3]
products gene name :
[PLOD3]
other gene names :
[PLOD3; PLOD3; LH3; LH3]
uniprot entry name :
PLOD3_HUMAN
clonality :
polyclonal
isotype :
IgG
host :
unknown
reactivity :
Human
sequence length :
738
purity :
Affinity Purification
concentration :
1mg/ml
storage stability :
Store at -20°C. Avoid freeze/thaw cycles.
tested application :
Western Blot (WB)
app notes :
WB: 1:200 - 1:2000
image1 heading :
Western Blot (WB)
other info1 :
Source: Rabbit. Immunogen: A synthetic peptide of human PLOD3
other info2 :
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
products categories :
Polyclonal
products description :
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.
ncbi gi num :
6093731
ncbi acc num :
O60568
uniprot acc num :
O60568
ncbi mol weight :
85kDa
ncbi pathways :
Collagen Biosynthesis And Modifying Enzymes Pathway (645289); Collagen Formation Pathway (645288); Extracellular Matrix Organization Pathway (576262); Lysine Degradation Pathway (82956); Lysine Degradation Pathway (320); Other Types Of O-glycan Biosynthesis Pathway (82978); Other Types Of O-glycan Biosynthesis Pathway (349)
ncbi summary :
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]
uniprot summary :
PLOD3: 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. Defects in PLOD3 are the cause of lysyl hydroxylase 3 deficiency (LH3 deficiency); 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. Protein type: Oxidoreductase; Endoplasmic reticulum; Amino Acid Metabolism - lysine degradation; EC 1.14.11.4. Chromosomal Location of Human Ortholog: 7q22. Cellular Component: endoplasmic reticulum membrane; endoplasmic reticulum; rough endoplasmic reticulum membrane. Molecular Function: protein binding; L-ascorbic acid binding; procollagen galactosyltransferase activity; procollagen-lysine 5-dioxygenase activity; iron ion binding; procollagen glucosyltransferase activity. Biological Process: neural tube development; extracellular matrix organization and biogenesis; protein localization; collagen fibril organization; in utero embryonic development; epidermis morphogenesis; protein modification process; endothelial cell morphogenesis; vasodilation; cellular response to hormone stimulus. Disease: Bone Fragility With Contractures, Arterial Rupture, And Deafness
size1 :
0.1 mL
price1 :
275 USD
size2 :
0.2 mL
price2 :
430
more info or order :
company information
MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com1-888-627-0165
headquarters: USA
MyBioSource, LLC was orginally founded in Vancouver by three enthusiastic scientists who are passionate about providing the world with the best reagents available. Together, they form a company with a big vision known as MyBioSource. MyBioSource is now located in San Diego, California, USA.
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