Next Article in Journal
Decreased R:FR Ratio in Incident White Light Affects the Composition of Barley Leaf Lipidome and Freezing Tolerance in a Temperature-Dependent Manner
Next Article in Special Issue
Hedging against Neuropathic Pain: Role of Hedgehog Signaling in Pathological Nerve Healing
Previous Article in Journal
Proposing Urothelial and Muscle In Vitro Cell Models as a Novel Approach for Assessment of Long-Term Toxicity of Nanoparticles
Previous Article in Special Issue
The Role of Smoothened in Cancer
Review

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

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://0-doi-org.brum.beds.ac.uk/10.3390/ijms21207559
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)
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. View Full-Text
Keywords: Gorlin syndrome; nerved basal cell carcinoma; hedgehog pathway Gorlin syndrome; nerved basal cell carcinoma; hedgehog pathway
Show Figures

Figure 1

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://0-doi-org.brum.beds.ac.uk/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://0-doi-org.brum.beds.ac.uk/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://0-doi-org.brum.beds.ac.uk/10.3390/ijms21207559

Find Other Styles
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Back to TopTop