Ontogeny of Phex/PHEX protein expression in mouse embryo and subcellular localization in osteoblasts

Rajiv Kumar, Y. Sabbagh, Joseph Peter Grande, P. C. Roche, M. K. Drezner, J. L. Salisbury, J. P. Grande, E. M. Poeschla, Jeffrey L Salisbury

Research output: Contribution to journalArticle

73 Citations (Scopus)

Abstract

PHEX, a phosphate-regulating gene with homologies to endopeptidases on the X chromosome, is mutated in X-linked hypophosphatemia (XLH) in humans and mice (Hyp). Although recent observations indicate that Phex protein is expressed primarily in bone and may play an important role in osteoblast function and bone mineralization, the pattern of the Phex protein expression in the developing skeleton and its subcellular localization in osteoblasts remain unknown. We examined the ontogeny of the Phex protein in the developing mouse embryo and its subcellular localization in osteoblasts using a specific antibody to the protein. Immunohistochemical staining of mouse embryos revealed expression of Phex in osteogenic precursors in developing vertebral bodies and developing long bones on day 16 postcoitum (pc) and thereafter. Calvaria from day 18 pc mice showed Phex epitopes in osteoblasts. No Phex immunoreactivity was detected in lung, heart, hepatocytes, kidney, intestine, skeletal muscle, or adipose tissue of mouse embryos. Interestingly, embryonic mouse skin showed moderate amounts of Phex immunostaining. In postnatal mice, Phex expression was observed in osteoblasts and osteocytes. Moderate expression of Phex was seen in odontoblasts and slight immunoreactivity was observed in ameloblasts. Confocal microscopy revealed the presence of immunoreactive PHEX protein in the Golgi apparatus and endoplasmic reticulum of osteoblasts from normal mice and in osteoblasts from Hyp mice transduced with a human PHEX viral expression vector. PHEX protein was not detected in untransduced Hyp osteoblasts. These data indicate that Phex protein is expressed in osteoblasts and osteocytes during the embryonic and postnatal periods and that within bone, Phex may be a unique marker for cells of the osteoblast/osteocyte lineage.

Original languageEnglish (US)
Pages (from-to)311-320
Number of pages10
JournalJournal of Bone and Mineral Research
Volume17
Issue number2
StatePublished - 2002

Fingerprint

Osteoblasts
Embryonic Structures
Proteins
Osteocytes
Bone and Bones
Familial Hypophosphatemic Rickets
Ameloblasts
Odontoblasts
Physiologic Calcification
Endopeptidases
X Chromosome
Golgi Apparatus
Skull
Skeleton
Confocal Microscopy
Endoplasmic Reticulum
Intestines
Adipose Tissue
Epitopes
Hepatocytes

Keywords

  • Antibody
  • Osteoblasts
  • Osteocytes
  • Phex/PHEX
  • X-linked hypophosphatemia

ASJC Scopus subject areas

  • Surgery

Cite this

Ontogeny of Phex/PHEX protein expression in mouse embryo and subcellular localization in osteoblasts. / Kumar, Rajiv; Sabbagh, Y.; Grande, Joseph Peter; Roche, P. C.; Drezner, M. K.; Salisbury, J. L.; Grande, J. P.; Poeschla, E. M.; Salisbury, Jeffrey L.

In: Journal of Bone and Mineral Research, Vol. 17, No. 2, 2002, p. 311-320.

Research output: Contribution to journalArticle

Kumar, R, Sabbagh, Y, Grande, JP, Roche, PC, Drezner, MK, Salisbury, JL, Grande, JP, Poeschla, EM & Salisbury, JL 2002, 'Ontogeny of Phex/PHEX protein expression in mouse embryo and subcellular localization in osteoblasts', Journal of Bone and Mineral Research, vol. 17, no. 2, pp. 311-320.
Kumar, Rajiv ; Sabbagh, Y. ; Grande, Joseph Peter ; Roche, P. C. ; Drezner, M. K. ; Salisbury, J. L. ; Grande, J. P. ; Poeschla, E. M. ; Salisbury, Jeffrey L. / Ontogeny of Phex/PHEX protein expression in mouse embryo and subcellular localization in osteoblasts. In: Journal of Bone and Mineral Research. 2002 ; Vol. 17, No. 2. pp. 311-320.
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AU - Kumar, Rajiv

AU - Sabbagh, Y.

AU - Grande, Joseph Peter

AU - Roche, P. C.

AU - Drezner, M. K.

AU - Salisbury, J. L.

AU - Grande, J. P.

AU - Poeschla, E. M.

AU - Salisbury, Jeffrey L

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AB - PHEX, a phosphate-regulating gene with homologies to endopeptidases on the X chromosome, is mutated in X-linked hypophosphatemia (XLH) in humans and mice (Hyp). Although recent observations indicate that Phex protein is expressed primarily in bone and may play an important role in osteoblast function and bone mineralization, the pattern of the Phex protein expression in the developing skeleton and its subcellular localization in osteoblasts remain unknown. We examined the ontogeny of the Phex protein in the developing mouse embryo and its subcellular localization in osteoblasts using a specific antibody to the protein. Immunohistochemical staining of mouse embryos revealed expression of Phex in osteogenic precursors in developing vertebral bodies and developing long bones on day 16 postcoitum (pc) and thereafter. Calvaria from day 18 pc mice showed Phex epitopes in osteoblasts. No Phex immunoreactivity was detected in lung, heart, hepatocytes, kidney, intestine, skeletal muscle, or adipose tissue of mouse embryos. Interestingly, embryonic mouse skin showed moderate amounts of Phex immunostaining. In postnatal mice, Phex expression was observed in osteoblasts and osteocytes. Moderate expression of Phex was seen in odontoblasts and slight immunoreactivity was observed in ameloblasts. Confocal microscopy revealed the presence of immunoreactive PHEX protein in the Golgi apparatus and endoplasmic reticulum of osteoblasts from normal mice and in osteoblasts from Hyp mice transduced with a human PHEX viral expression vector. PHEX protein was not detected in untransduced Hyp osteoblasts. These data indicate that Phex protein is expressed in osteoblasts and osteocytes during the embryonic and postnatal periods and that within bone, Phex may be a unique marker for cells of the osteoblast/osteocyte lineage.

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KW - Osteocytes

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