نتایج جستجو برای: microdeletion

تعداد نتایج: 1516  

Journal: :Cytogenetic and genome research 2010
P N Rao W Li L E L M Vissers J A Veltman R A Ophoff

The chromosomal band 17q21.31, containing the microtubule-associated protein tau (MAPT) gene, is a hotspot for chromosomal rearrangements. It is known to contain a common inversion polymorphism of approximately 900 kb in populations with European ancestry. The inverted configuration is linked to a distinct MAPT haplotype, H2, which is relatively common in Europeans but nearly absent in Asian an...

Journal: :Journal of medical genetics 2000
P Blanco M Shlumukova C A Sargent M A Jobling N Affara M E Hurles

The Y chromosome provides a unique opportunity to study mutational processes within the human genome, decoupled from the confounding effects of interchromosomal recombination. It has been suggested that the increased density of certain dispersed repeats on the Y could account for the high frequency of causative microdeletions relative to single nucleotide mutations in infertile males. Previousl...

Journal: :Archives of Iranian medicine 2014
Houra Loghmani Khouzani Ariana Kariminejad Gholamreza Zamani Maryam Ghalandary Bita Bozorgmehr Susan Amirsalari Faezeh Mojahedi Sayed Hassan Tonekaboni Roxana Kariminejad Hossein Najmabadi

BACKGROUND Intellectual Disabilities (ID), defined as a state of developmental deficit, result in significant limitation of intellect and poor adaptation behavior. A number of genetic factors can result in ID, such as chromosomal abnormalities, copy number variation, and single gene defect. Karyotyping is the routine method for detecting chromosomal abnormalities in patients with ID. More recen...

Journal: :Archives of disease in childhood 2003
K L Greenhalgh I A Aligianis G Bromilow H Cox C Hill Y Stait B J Leech P W Lunt M Ellis

AIM To draw up recommendations for the investigation and management of children with a microdeletion of chromosome 22q11. METHODS A retrospective review of case notes from patients with a chromosome 22q11 microdeletion identified by cytogenetics laboratories of the south and west of Britain over a four year period. RESULTS A total of 210 cases were identified. Age at diagnosis was 0-1 years...

Journal: :The Journal of clinical investigation 2003
Murat Bastepe Leopold F Fröhlich Geoffrey N Hendy Olafur S Indridason Robert G Josse Hiroyuki Koshiyama Jarmo Körkkö Jon M Nakamoto Arlan L Rosenbloom Arnold H Slyper Toshitsugu Sugimoto Agathocles Tsatsoulis John D Crawford Harald Jüppner

Patients with pseudohypoparathyroidism type Ib (PHP-Ib) have hypocalcemia and hyperphosphatemia due to renal parathyroid hormone (PTH) resistance, but lack physical features of Albright hereditary osteodystrophy. PHP-Ib is thus distinct from PHP-Ia, which is caused by mutations in the GNAS exons encoding the G protein alpha subunit. However, an imprinted autosomal dominant form of PHP-Ib (AD-PH...

2013
Margarita G. Todorova Matthias C. Grieshaber Rafael J. A. Cámara Peter Miny Anja M. Palmowski-Wolfe Margarita Georgieva Todorova

Background: Williams-Beuren syndrome is characterized by mild mental retardation, specific neurocognitive profile, hypercalcemia during infancy, distinctive facial features and cardiovascular diseases. We report on complete ophthalmologic, sonographic and genetic evaluation of a girl with a clinical phenotype of WilliamsBeuren syndrome, associated with unilateral anterior segment dysgenesis and...

2013
Ramaswamy Suganthi VV Vijesh Sanjay Jayachandran Jahangir Ali Fathima Benazir

BACKGROUND Y chromosomal microdeletion is an important genetic disorder, which may arise due to intrachromosomal recombination between homologous sequences in the male specific region of the human Y chromosome. It is frequently associated with the quantitative reduction of sperm. The screening for Y chromosomal microdeletions has a great clinical value. OBJECTIVE To develop a sequence tagged ...

Journal: :Clinical genetics 2016
X Ning S Farschtschi A Jones H Kehrer-Sawatzki V-F Mautner J M Friedman

Microdeletions of the entire NF1 gene and surrounding genomic region occur in about 5% of patients with neurofibromatosis 1 (NF1). NF1 microdeletion patients usually have more cutaneous and plexiform neurofibromas and a higher risk of developing malignant peripheral nerve sheath tumors than other people with NF1. Somatic overgrowth has also been observed in NF1 microdeletion patients, an observ...

Journal: :Journal of medical genetics 2008
D A Koolen A J Sharp J A Hurst H V Firth S J L Knight A Goldenberg P Saugier-Veber R Pfundt L E L M Vissers A Destrée B Grisart L Rooms N Van der Aa M Field A Hackett K Bell M J M Nowaczyk G M S Mancini P J Poddighe C E Schwartz E Rossi M De Gregori L L Antonacci-Fulton M D McLellan J M Garrett M A Wiechert T L Miner S Crosby R Ciccone L Willatt A Rauch M Zenker S Aradhya M A Manning T M Strom J Wagenstaller A C Krepischi-Santos A M Vianna-Morgante C Rosenberg S M Price H Stewart C Shaw-Smith H G Brunner A O M Wilkie J A Veltman O Zuffardi E E Eichler B B A de Vries

BACKGROUND The chromosome 17q21.31 microdeletion syndrome is a novel genomic disorder that has originally been identified using high resolution genome analyses in patients with unexplained mental retardation. AIM We report the molecular and/or clinical characterisation of 22 individuals with the 17q21.31 microdeletion syndrome. RESULTS We estimate the prevalence of the syndrome to be 1 in 1...

Journal: :Turk pediatri arsivi 2014
Murat Özkale İlknur Erol

22q11.2 microdeletion which involves DiGeorge syndrome, velo-cardiofacial syndrome and conotruncal anomaly face syndrome occurs as a result of a deletion in the short segment of the long arm of the 22th chromosome. Patients with this syndrome have a wide clinical spectrum including learning difficulty, dysmorphic face, cardiac anomalies, hypocalcemia, hypoparathyroidism, cleft palate, thymus an...

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