Entity Details

Primary name PHEX_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP78562
EntryNamePHEX_HUMAN
FullNamePhosphate-regulating neutral endopeptidase PHEX
TaxID9606
Evidenceevidence at protein level
Length749
SequenceStatuscomplete
DateCreated1997-11-01
DateModified2021-06-02

Ontological Relatives

GenesPHEX

GO terms

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GOName
GO:0001501 skeletal system development
GO:0004222 metalloendopeptidase activity
GO:0005783 endoplasmic reticulum
GO:0005794 Golgi apparatus
GO:0005886 plasma membrane
GO:0005887 integral component of plasma membrane
GO:0006464 cellular protein modification process
GO:0006508 proteolysis
GO:0007267 cell-cell signaling
GO:0008270 zinc ion binding
GO:0016485 protein processing
GO:0019637 organophosphate metabolic process
GO:0030282 bone mineralization
GO:0030324 lung development
GO:0042476 odontogenesis
GO:0048471 perinuclear region of cytoplasm
GO:0060348 bone development
GO:0060416 response to growth hormone
GO:0071305 cellular response to vitamin D
GO:0071374 cellular response to parathyroid hormone stimulus
GO:1904383 response to sodium phosphate
GO:1990418 response to insulin-like growth factor stimulus

Subcellular Location

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Subcellular Location
Cell membrane

Domains

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DomainNameCategoryType
IPR000718 Peptidase M13FamilyFamily
IPR008753 Peptidase M13, N-terminal domainDomainDomain
IPR018497 Peptidase M13, C-terminal domainDomainDomain
IPR024079 Metallopeptidase, catalytic domain superfamilyFamilyHomologous superfamily
IPR042089 Peptidase M13, domain 2FamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
307800 OMIMHypophosphatemic rickets, X-linked dominant (XLHR)A 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. The disease is caused by variants affecting the gene represented in this entry.

Interactions

2 interactions

InteractorPartnerSourcesPublicationsLink
PHEX_HUMANMEPE_HUMANUniProt15664000 details
PHEX_HUMANFGF23_HUMANHPRD11409890 details