scholarly journals Microcephalin and pericentrin regulate mitotic entry via centrosome-associated Chk1

2009 ◽  
Vol 185 (7) ◽  
pp. 1149-1157 ◽  
Author(s):  
Alexandra Tibelius ◽  
Joachim Marhold ◽  
Hanswalter Zentgraf ◽  
Christoph E. Heilig ◽  
Heidemarie Neitzel ◽  
...  
Keyword(s):  
Ataxia Telangiectasia ◽  
Cyclin B ◽  
Fetal Life ◽  
Type Ii ◽  
Primary Microcephaly ◽  
Seckel Syndrome ◽  
Mitotic Entry ◽  
Primordial Dwarfism

Primary microcephaly, Seckel syndrome, and microcephalic osteodysplastic primordial dwarfism type II (MOPD II) are disorders exhibiting marked microcephaly, with small brain sizes reflecting reduced neuron production during fetal life. Although primary microcephaly can be caused by mutations in microcephalin (MCPH1), cells from patients with Seckel syndrome and MOPD II harbor mutations in ataxia telangiectasia and Rad3 related (ATR) or pericentrin (PCNT), leading to disturbed ATR signaling. In this study, we show that a lack of MCPH1 or PCNT results in a loss of Chk1 from centrosomes with subsequently deregulated activation of centrosomal cyclin B–Cdk1.

Majewski osteodysplastic primordial dwarfism type II (MOPD II) syndrome previously diagnosed as Seckel syndrome: Report of a novel mutation of thePCNTgene

2009 ◽  
Vol 149A (11) ◽  
pp. 2452-2456 ◽  
Author(s):  
Maria Piane ◽  
Matteo Della Monica ◽  
Gianluca Piatelli ◽  
Patrizia Lulli ◽  
Fortunato Lonardo ◽  
...  
Keyword(s):  
Novel Mutation ◽  
Type Ii ◽  
Seckel Syndrome ◽  
Primordial Dwarfism ◽  
Syndrome Report

Identification of three novel mutations in PCNT in vietnamese patients with microcephalic osteodysplastic primordial dwarfism type II

2021 ◽  
Vol 43 (2) ◽  
pp. 115-121
Author(s):  
Thu Hien Nguyen ◽  
Ngoc-Lan Nguyen ◽  
Chi Dung Vu ◽  
Can Thi Bich Ngoc ◽  
Ngoc Khanh Nguyen ◽  
...  
Keyword(s):  
Type Ii ◽  
Novel Mutations ◽  
Primordial Dwarfism

Fibroblast-pneumonocyte factor

1989 ◽  
Vol 257 (4) ◽  
pp. L174-L178 ◽  
Author(s):  
B. T. Smith ◽  
M. Post
Keyword(s):  
Physiological Role ◽  
Fetal Lung ◽  
Lung Fibroblast ◽  
Cell Phenotype ◽  
Alveolar Type ◽  
Fetal Life ◽  
Type Ii ◽  
Lung Maturation ◽  
Organ Specific ◽  
Type Ii Cell

Fibroblast-pneumonocyte factor (FPF) is an organ-specific differentiation factor produced, under glucocorticoid regulation, by the fetal lung fibroblast, which in turn enhances the differentiation of the alveolar type II cell with respect to surfactant production. In this article we review the appearance of the type II cell phenotype during fetal life and the action of FPF to time full acquisition of this phenotype. Evidence supporting a physiological role for this factor is reviewed as are details of its production by the fetal lung fibroblast and action on the type II cell. Finally we summarize possible future clinical advantages of using such a material to regulate lung maturation compared with currently used maternal glucocorticoid therapy. These include a more rapid effect and bypassing the inhibition of glucocorticoid action seen in male fetuses and in infants of diabetic mothers.

A new case of the osteodysplastic primordial dwarfism type II

1987 ◽  
Vol 26 (4) ◽  
pp. 819-824 ◽  
Author(s):  
Patrick J. Willems ◽  
Catrienus Rouwé ◽  
G. Peter A. Smit ◽  
John M. Opitz ◽  
James F. Reynolds
Keyword(s):  
Type Ii ◽  
Primordial Dwarfism

Primary microcephaly, primordial dwarfism, and brachydactyly in adult cases with biallelic skipping of RTTN exon 42

2019 ◽  
Author(s):  
Muhammad Zakaria ◽  
Ambrin Fatima ◽  
Joakim Klar ◽  
Johan Wikström ◽  
Uzma Abdullah ◽  
...  
Keyword(s):  
Primary Microcephaly ◽  
Primordial Dwarfism

scholarly journals The decision to enter mitosis: feedback and redundancy in the mitotic entry network

2009 ◽  
Vol 185 (2) ◽  
pp. 193-202 ◽  
Author(s):  
Arne Lindqvist ◽  
Verónica Rodríguez-Bravo ◽  
René H. Medema
Keyword(s):  
Cell Cycle ◽  
Normal Cell ◽  
Cell Cycle Progression ◽  
Feedback Loops ◽  
Cyclin B ◽  
Cycle Progression ◽  
Mitotic Entry ◽  
Different Levels ◽  
Normal Cell Cycle

The decision to enter mitosis is mediated by a network of proteins that regulate activation of the cyclin B–Cdk1 complex. Within this network, several positive feedback loops can amplify cyclin B–Cdk1 activation to ensure complete commitment to a mitotic state once the decision to enter mitosis has been made. However, evidence is accumulating that several components of the feedback loops are redundant for cyclin B–Cdk1 activation during normal cell division. Nonetheless, defined feedback loops become essential to promote mitotic entry when normal cell cycle progression is perturbed. Recent data has demonstrated that at least three Plk1-dependent feedback loops exist that enhance cyclin B–Cdk1 activation at different levels. In this review, we discuss the role of various feedback loops that regulate cyclin B–Cdk1 activation under different conditions, the timing of their activation, and the possible identity of the elusive trigger that controls mitotic entry in human cells.

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