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NHERF1/EBP50 is an organizer of polarity structures and a diagnostic marker in ependymoma.

Publication ,  Journal Article
Georgescu, M-M; Yell, P; Mobley, BC; Shang, P; Georgescu, T; Wang, S-HJ; Canoll, P; Hatanpaa, KJ; White, CL; Raisanen, JM
Published in: Acta Neuropathol Commun
March 8, 2015

NHERF1/EBP50, an adaptor protein required for epithelial morphogenesis, has been implicated in the progression of various human malignancies. NHERF1-deficient mice have intestinal brush border structural defects and we report here that they also have disorganized ependymal cilia with development of non-obstructive hydrocephalus. Examination of mouse and human brain tissues revealed highest NHERF1 expression at the apical plasma membrane of ependymal cells. In ependymal tumors, NHERF1 expression was retained in polarized membrane structures, such as microlumens, rosettes and canals, where it co-localized with some of its ligands, such as moesin and PTEN. Analysis of a comprehensive panel of 113 tumors showed robust NHERF1 labeling of microlumens in 100% of ependymomas, subependymomas, and pediatric anaplastic ependymomas, and in 67% of adult anaplastic ependymomas. NHERF1 staining was present in 35% of ependymoma cases that lacked reactivity for EMA, the routine immunohistochemical marker used for ependymoma diagnosis. NHERF1 labeling of microlumens was either absent or rarely seen in other types of brain tumors analyzed, denoting NHERF1 as a reliable diagnostic marker of ependymal tumors. Anaplastic foci and a subset of adult anaplastic ependymomas showed complete absence of NHERF1-labeled polarity structures, consistent with a loss of differentiation in these aggressive tumors. These data highlight a role for NHERF1 in ependymal morphogenesis with direct application to the diagnosis of ependymal tumors.

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Published In

Acta Neuropathol Commun

DOI

EISSN

2051-5960

Publication Date

March 8, 2015

Volume

3

Start / End Page

11

Location

England

Related Subject Headings

  • beta Catenin
  • Sodium-Hydrogen Exchangers
  • Receptor, Platelet-Derived Growth Factor alpha
  • Protein Tyrosine Phosphatases
  • Phosphoproteins
  • Nerve Tissue Proteins
  • Microfilament Proteins
  • Mice, Transgenic
  • Mice, Inbred C57BL
  • Mice
 

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Georgescu, M.-M., Yell, P., Mobley, B. C., Shang, P., Georgescu, T., Wang, S.-H., … Raisanen, J. M. (2015). NHERF1/EBP50 is an organizer of polarity structures and a diagnostic marker in ependymoma. Acta Neuropathol Commun, 3, 11. https://doi.org/10.1186/s40478-015-0197-z
Georgescu, Maria-Magdalena, Paul Yell, Bret C. Mobley, Ping Shang, Theodora Georgescu, Shih-Hsiu J. Wang, Peter Canoll, Kimmo J. Hatanpaa, Charles L. White, and Jack M. Raisanen. “NHERF1/EBP50 is an organizer of polarity structures and a diagnostic marker in ependymoma.Acta Neuropathol Commun 3 (March 8, 2015): 11. https://doi.org/10.1186/s40478-015-0197-z.
Georgescu M-M, Yell P, Mobley BC, Shang P, Georgescu T, Wang S-HJ, et al. NHERF1/EBP50 is an organizer of polarity structures and a diagnostic marker in ependymoma. Acta Neuropathol Commun. 2015 Mar 8;3:11.
Georgescu, Maria-Magdalena, et al. “NHERF1/EBP50 is an organizer of polarity structures and a diagnostic marker in ependymoma.Acta Neuropathol Commun, vol. 3, Mar. 2015, p. 11. Pubmed, doi:10.1186/s40478-015-0197-z.
Georgescu M-M, Yell P, Mobley BC, Shang P, Georgescu T, Wang S-HJ, Canoll P, Hatanpaa KJ, White CL, Raisanen JM. NHERF1/EBP50 is an organizer of polarity structures and a diagnostic marker in ependymoma. Acta Neuropathol Commun. 2015 Mar 8;3:11.
Journal cover image

Published In

Acta Neuropathol Commun

DOI

EISSN

2051-5960

Publication Date

March 8, 2015

Volume

3

Start / End Page

11

Location

England

Related Subject Headings

  • beta Catenin
  • Sodium-Hydrogen Exchangers
  • Receptor, Platelet-Derived Growth Factor alpha
  • Protein Tyrosine Phosphatases
  • Phosphoproteins
  • Nerve Tissue Proteins
  • Microfilament Proteins
  • Mice, Transgenic
  • Mice, Inbred C57BL
  • Mice