Cell Biology Antibodies 2
Anti-PLOD3 Antibody (CAB10547)
- SKU:
- CAB10547
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Description
Antibody Name: | Anti-PLOD3 Antibody |
Antibody SKU: | CAB10547 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB |
Reactivity: | Human, Mouse, Rat |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 25-280 of human PLOD3 (NP_001075.1). |
Application: | WB |
Recommended Dilution: | WB 1:500 - 1:2000 |
Reactivity: | Human, Mouse, Rat |
Positive Samples: | MCF7, SW480, NCI-H460, 22Rv1, HepG2, Mouse lung, Rat liver |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 25-280 of human PLOD3 (NP_001075.1). |
Purification Method: | Affinity purification |
Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Isotype: | IgG |
Sequence: | SDRP RGRD PVNP EKLL VITV ATAE TEGY LRFL RSAE FFNY TVRT LGLG EEWR GGDV ARTV GGGQ KVRW LKKE MEKY ADRE DMII MFVD SYDV ILAG SPTE LLKK FVQS GSRL LFSA ESFC WPEW GLAE QYPE VGTG KRFL NSGG FIGF ATTI HQIV RQWK YKDD DDDQ LFYT RLYL DPGL REKL SLNL DHKS RIFQ NLNG ALDE VVLK FDRN RVRI RNVA YDTL PIVV HGNG PTKL QLNY LGNY VPNG WTPE GGCG |
Gene ID: | 8985 |
Uniprot: | O60568 |
Cellular Location: | Lumenal side, Peripheral membrane protein, Rough endoplasmic reticulum membrane |
Calculated MW: | 84kDa |
Observed MW: | 80kDa |
Synonyms: | PLOD3, LH3 |
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. |
UniProt Protein Function: | 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. |
UniProt Protein Details: | Protein type:Amino Acid Metabolism - lysine degradation; EC 1.14.11.4; Endoplasmic reticulum; Oxidoreductase Chromosomal Location of Human Ortholog: 7q22.1 Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; Golgi apparatus Molecular Function:procollagen galactosyltransferase activity; procollagen glucosyltransferase activity; procollagen-lysine 5-dioxygenase activity; protein binding Biological Process: hydroxylysine biosynthetic process; peptidyl-lysine hydroxylation; protein amino acid O-linked glycosylation Disease: Bone Fragility With Contractures, Arterial Rupture, And Deafness |
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 Code: | O60568 |
NCBI GenInfo Identifier: | 6093731 |
NCBI Gene ID: | 8985 |
NCBI Accession: | O60568.1 |
UniProt Secondary Accession: | O60568,Q540C3, B2R6W6, |
UniProt Related Accession: | O60568 |
Molecular Weight: | 85kDa |
NCBI Full Name: | Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3 |
NCBI Synonym Full Names: | procollagen-lysine,2-oxoglutarate 5-dioxygenase 3 |
NCBI Official Symbol: | PLOD3 |
NCBI Official Synonym Symbols: | LH3 |
NCBI Protein Information: | procollagen-lysine,2-oxoglutarate 5-dioxygenase 3 |
UniProt Protein Name: | Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3 |
UniProt Synonym Protein Names: | Lysyl hydroxylase 3; LH3 |
Protein Family: | Procollagen-lysine,2-oxoglutarate 5-dioxygenase |
UniProt Gene Name: | PLOD3 |
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