PHEX Antibody (PACO59840)
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주문- SKU:
- PACO59840
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | PHEX Antibody (PACO59840) |
Antibody SKU: | PACO59840 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, IF |
Recommended Dilutions: | ELISA:1:2000-1:10000, IF:1:50-1:200 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Phosphate-regulating neutral endopeptidase protein (524-673AA) |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
Purification Method: | >95%, Protein G purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Immunofluorescence staining of HepG2 cells with PACO59840 at 1:133, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
Background: | Probably involved in bone and dentin mineralization and renal phosphate reabsorption. |
Synonyms: | Phosphate-regulating neutral endopeptidase (EC 3.4.24) (Metalloendopeptidase homolog PEX) (Vitamin D-resistant hypophosphatemic rickets protein) (X-linked hypophosphatemia protein) (HYP), PHEX, PEX |
UniProt Protein Function: | PHEX: Probably involved in bone and dentin mineralization and renal phosphate reabsorption. Defects in PHEX are a cause of hypophosphatemic rickets, X-linked dominant (XLHR). XLHR is an X-linked dominant disorder characterized by impaired phosphate uptake in the kidney, which is likely to be caused by abnormal regulation of sodium phosphate cotransport in the proximal tubules. Clinical manifestations include skeletal deformities, growth failure, craniosynostosis, paravertebral calcifications, pseudofractures in lower extremities, and muscular hypotonia with onset in early childhood. X-linked hypophosphatemic rickets is the most common form of hypophosphatemia with an incidence of 1 in 20000. Belongs to the peptidase M13 family. |
UniProt Protein Details: | Protein type:Membrane protein, integral; Protease; EC 3.4.24.- Chromosomal Location of Human Ortholog: Xp22.2-p22.1 Cellular Component: integral to plasma membrane; plasma membrane Molecular Function:zinc ion binding Biological Process: cell-cell signaling; protein modification process; proteolysis; skeletal development Disease: Hypophosphatemic Rickets, X-linked Dominant |
NCBI Summary: | The protein encoded by this gene is a transmembrane endopeptidase that belongs to the type II integral membrane zinc-dependent endopeptidase family. The protein is thought to be involved in bone and dentin mineralization and renal phosphate reabsorption. Mutations in this gene cause X-linked hypophosphatemic rickets. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2013] |
UniProt Code: | P78562 |
NCBI GenInfo Identifier: | 2499917 |
NCBI Gene ID: | 5251 |
NCBI Accession: | P78562.1 |
UniProt Secondary Accession: | P78562,O00678, Q13646, Q2M325, Q93032, Q99827, |
UniProt Related Accession: | P78562 |
Molecular Weight: | 86,474 Da |
NCBI Full Name: | Phosphate-regulating neutral endopeptidase |
NCBI Synonym Full Names: | phosphate regulating endopeptidase homolog, X-linked |
NCBI Official Symbol: | PHEX |
NCBI Official Synonym Symbols: | HYP; PEX; XLH; HPDR; HYP1; LXHR; HPDR1 |
NCBI Protein Information: | phosphate-regulating neutral endopeptidase |
UniProt Protein Name: | Phosphate-regulating neutral endopeptidase |
UniProt Synonym Protein Names: | Metalloendopeptidase homolog PEX; Vitamin D-resistant hypophosphatemic rickets protein; X-linked hypophosphatemia protein; HYP |
Protein Family: | Phosphate-regulating neutral endopeptidase |
UniProt Gene Name: | PHEX |
UniProt Entry Name: | PHEX_HUMAN |
Antibodies |
Anti-PHEX Antibody (CAB7918) |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
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Anti-FITC Goat Anti-Rabbit IgG (H+L) Antibody (CABS011) |
Anti-HRP-conjugated Beta Actin Antibody (CABC028) |