نتایج جستجو برای: autosomal recessive primary microcephaly

تعداد نتایج: 682552  

Journal: :Human molecular genetics 2005
Natalay Kouprina Adam Pavlicek N Keith Collins Megumi Nakano Vladimir N Noskov Jun-Ichirou Ohzeki Ganeshwaran H Mochida John I Risinger Paul Goldsmith Michelle Gunsior Greg Solomon William Gersch Jung-Hyun Kim J Carl Barrett Christopher A Walsh Jerzy Jurka Hiroshi Masumoto Vladimir Larionov

The most common cause of primary autosomal recessive microcephaly (MCPH) appears to be mutations in the ASPM gene which is involved in the regulation of neurogenesis. The predicted gene product contains two putative N-terminal calponin-homology (CH) domains and a block of putative calmodulin-binding IQ domains common in actin binding cytoskeletal and signaling proteins. Previous studies in mous...

2018
Majid Alfadhel Marwan Nashabat Khalid Hundallah Amal Al Hashem Ahmed Alrumayyan Brahim Tabarki

PRUNE syndrome, or neurodevelopmental disorder with microcephaly, hypotonia, and variable brain anomalies (OMIM#617481), is a new rare autosomal recessive neurodevelopmental disease that is caused by homozygous or compound heterozygous mutation in PRUNE1 on chromosome 1q21. Here, We report on 12-month-old and 30-month-old girls from 2 unrelated Saudi families with typical presentations of PRUNE...

Journal: :Developmental cell 2010
Jose A Barrera Ling-Rong Kao Robert E Hammer Joachim Seemann Jannon L Fuchs Timothy L Megraw

Centriole duplication occurs once per cell cycle, ensuring that each cell contains two centrosomes, each containing a mother-daughter pair of tightly engaged centrioles at mitotic entry. Loss of the tight engagement between mother and daughter centrioles appears to license the next round of centriole duplication. However, the molecular mechanisms regulating this process remain largely unknown. ...

Journal: :international journal of pediatrics 0
shruti saraswat department of paediatrics, hi tech medical college, utkal university, bhubaneswar, odisha, india. satish mohanty department of paediatrics, hi tech medical college, utkal university, bhubaneswar, odisha, india.

background robinow syndrome is a rare congenital disorder with phenotypically heterogeneous abnormalities. two modes of inheritances are known for this syndrome namely autosomal recessive and autosomal dominant. case report we describe here an eighteen-month-old child who had mesomelic short stature, abnormal facial features, clinodactyly, micropenis and vertebral changes which were further sup...

Journal: :basic and clinical neuroscience 0
sunil gothwal department of pediatrics, all india institute of medical science, new delhi, india. swati nayan sms mc

hallervorden-spatz syndrome is a disorder characterized by dystonia, parkinsonism, and iron accumulation in the brain. the disease is caused by mutations in gene encoding pantothenate kinase 2 (pank2) and patients have pantothenate kinase-associated neurodegeneration. we present an 8-year-old boy with progressive muscle dystonia, neuroregression, frequent fall and multiple injury marks of diffe...

2001
Wolfgang Ketterle

We summarize all experimental studies of Bose-Einstein condensation (BEC) in dilute atomic gases reported thus far and discuss the experimental techniques used to produce, manipulate and observe nanokelvin samples of atoms.

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