Cell Biology Antibodies 10

Anti-PLOD2 Antibody (CAB6946)

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SKU:
CAB6946
Product Type:
Antibody
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-PLOD2 Antibody
Antibody SKU:CAB6946
Antibody Size:20uL, 50uL, 100uL
Application:WB IF
Reactivity:Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 470-650 of human PLOD2 (NP_891988.1).
Application:WB IF
Recommended Dilution:WB 1:500 - 1:2000 IF 1:50 - 1:100
Reactivity:Rat
Positive Samples:Rat kidney
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 470-650 of human PLOD2 (NP_891988.1).
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:KTLR SEMN ERNY FVRD KLDP DMAL CRNA REMT LQRE KDSP TPET FQML SPPK GVFM YISN RHEF GRLL STAN YNTS HYNN DLWQ IFEN PVDW KEKY INRD YSKI FTEN IVEQ PCPD VFWF PIFS EKAC DELV EEME HYGK WSGG KHHD SRIS GGYE NVPT DDIH MKQV DLEN VWLH FIRE F
Gene ID:5352
Uniprot:O00469
Cellular Location:Lumenal side, Peripheral membrane protein, Rough endoplasmic reticulum membrane
Calculated MW:49kDa/84kDa/87kDa
Observed MW:85kDa
Synonyms:PLOD2, BRKS2, LH2, TLH
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. Mutations in the coding region of this gene are associated with Bruck syndrome. Alternative splicing results in multiple transcript variants encoding different isoforms.
UniProt Protein Function:PLOD2: 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 PLOD2 are the cause of Bruck syndrome type 2 (BRKS2). Bruck syndrome, also known as osteogenesis imperfecta with congenital joint contractures, is an autosomal recessive disease characterized by generalized osteopenia, joint contractures at birth, fragile bones and short stature. It can be distinguished from osteogenesis imperfecta by the absence of hearing loss and dentinogenesis imperfecta, and by the presence of clubfoot and congenital joint limitations. The molecular defect is an aberrant cross-linking of bone collagen, due to underhydroxylation of lysine residues within the telopeptides of type I collagen, whereas the lysine residues in the triple helix are normal. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Endoplasmic reticulum; Amino Acid Metabolism - lysine degradation; Oxidoreductase; EC 1.14.11.4

Chromosomal Location of Human Ortholog: 3q24

Cellular Component: endoplasmic reticulum membrane; endoplasmic reticulum; rough endoplasmic reticulum membrane

Molecular Function:L-ascorbic acid binding; iron ion binding; procollagen-lysine 5-dioxygenase activity

Biological Process: extracellular matrix organization and biogenesis; response to hypoxia; protein modification process

Disease: Bruck Syndrome 2

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. Mutations in the coding region of this gene are associated with Bruck syndrome. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]
UniProt Code:O00469
NCBI GenInfo Identifier:62906878
NCBI Gene ID:5352
NCBI Accession:O00469.2
UniProt Related Accession:O00469
Molecular Weight:~ 85kDa
NCBI Full Name:Procollagen-lysine,2-oxoglutarate 5-dioxygenase 2
NCBI Synonym Full Names:procollagen-lysine,2-oxoglutarate 5-dioxygenase 2
NCBI Official Symbol:PLOD2  
NCBI Official Synonym Symbols:LH2; TLH; BRKS2  
NCBI Protein Information:procollagen-lysine,2-oxoglutarate 5-dioxygenase 2
UniProt Protein Name:Procollagen-lysine,2-oxoglutarate 5-dioxygenase 2
UniProt Synonym Protein Names:Lysyl hydroxylase 2; LH2
Protein Family:Procollagen-lysine,2-oxoglutarate 5-dioxygenase
UniProt Gene Name:PLOD2  
UniProt Entry Name:PLOD2_HUMAN
Anti-PLOD2 Antibody (CAB6946)Western blot analysis of extracts of rat kidney, using PLOD2 Antibody (CAB6946) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
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