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Volume 21
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Review

Gorlin Syndrome: Recent Advances in Genetic Testing and Molecular and Cellular Biological Research

by
Shoko Onodera
1,
Yuriko Nakamura
2 and
Toshifumi Azuma
1,*
1
Department of Biochemistry, Tokyo Dental College, 2-9-18 Kandamisaki-cho Chiyoda-ku, Tokyo 101-0061, Japan
2
Department of Oral Oncology, Oral and Maxillofacial Surgery, Tokyo Dental College, 5-11-13 Sugano, Ichikawa, Chiba 272-8513, Japan
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2020, 21(20), 7559; https://doi.org/10.3390/ijms21207559
Submission received: 2 September 2020 / Revised: 5 October 2020 / Accepted: 6 October 2020 / Published: 13 October 2020
(This article belongs to the Special Issue Hedgehog Signaling 2.0)
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Abstract

Gorlin syndrome is a skeletal disorder caused by a gain of function mutation in Hedgehog (Hh) signaling. The Hh family comprises of many signaling mediators, which, through complex mechanisms, play several important roles in various stages of development. The Hh information pathway is essential for bone tissue development. It is also the major driver gene in the development of basal cell carcinoma and medulloblastoma. In this review, we first present the recent advances in Gorlin syndrome research, in particular, the signaling mediators of the Hh pathway and their functions at the genetic level. Then, we discuss the phenotypes of mutant mice and Hh signaling-related molecules in humans revealed by studies using induced pluripotent stem cells.
Keywords: Gorlin syndrome; nerved basal cell carcinoma; hedgehog pathway Gorlin syndrome; nerved basal cell carcinoma; hedgehog pathway

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MDPI and ACS Style

Onodera, S.; Nakamura, Y.; Azuma, T. Gorlin Syndrome: Recent Advances in Genetic Testing and Molecular and Cellular Biological Research. Int. J. Mol. Sci. 2020, 21, 7559. https://doi.org/10.3390/ijms21207559

AMA Style

Onodera S, Nakamura Y, Azuma T. Gorlin Syndrome: Recent Advances in Genetic Testing and Molecular and Cellular Biological Research. International Journal of Molecular Sciences. 2020; 21(20):7559. https://doi.org/10.3390/ijms21207559

Chicago/Turabian Style

Onodera, Shoko, Yuriko Nakamura, and Toshifumi Azuma. 2020. "Gorlin Syndrome: Recent Advances in Genetic Testing and Molecular and Cellular Biological Research" International Journal of Molecular Sciences 21, no. 20: 7559. https://doi.org/10.3390/ijms21207559

APA Style

Onodera, S., Nakamura, Y., & Azuma, T. (2020). Gorlin Syndrome: Recent Advances in Genetic Testing and Molecular and Cellular Biological Research. International Journal of Molecular Sciences, 21(20), 7559. https://doi.org/10.3390/ijms21207559

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