Literature DB >> 26453364

Further Delineation of the ALG9-CDG Phenotype.

Sarah AlSubhi1, Amal AlHashem2, Anas AlAzami3, Kalthoum Tlili4, Saad AlShahwan1, Dirk Lefeber5, Fowzan S Alkuraya3,6, Brahim Tabarki7.   

Abstract

ALG9-CDG is one of the less frequently reported types of CDG. Here, we summarize the features of six patients with ALG9-CDG reported in the literature and report the features of four additional patients. The patients presented with drug-resistant infantile epilepsy, hypotonia, dysmorphic features, failure to thrive, global developmental disability, and skeletal dysplasia. One patient presented with nonimmune hydrops fetalis. A brain MRI revealed global atrophy with delayed myelination. Exome sequencing identified a novel homozygous mutation c.1075G>A, p.E359K of the ALG9 gene. The results of our analysis of these patients expand the knowledge of ALG9-CDG phenotype.

Entities:  

Keywords:  ALG9-CDG; Congenital disorders of glycosylation; Epilepsy; Hydrops fetalis; Skeletal dysplasia; Whole exome sequencing

Year:  2015        PMID: 26453364      PMCID: PMC4867846          DOI: 10.1007/8904_2015_504

Source DB:  PubMed          Journal:  JIMD Rep        ISSN: 2192-8304


  14 in total

1.  Congenital disorder of glycosylation type Ia presenting with hydrops fetalis.

Authors:  J M van de Kamp; D J Lefeber; G J G Ruijter; S J Steggerda; N S den Hollander; S M Willems; G Matthijs; B J H M Poorthuis; R A Wevers
Journal:  J Med Genet       Date:  2006-12-08       Impact factor: 6.318

2.  A lethal osteochondrodysplasia with mesomelic brachymelia, round pelvis, and congenital hepatic fibrosis: two siblings born to consanguineous parents.

Authors:  G Nishimura; M Nakayama; Y Fuke; N Suehara
Journal:  Pediatr Radiol       Date:  1998-01

Review 3.  Congenital disorders of glycosylation.

Authors:  J Jaeken
Journal:  Handb Clin Neurol       Date:  2013

4.  CDG-IL: an infant with a novel mutation in the ALG9 gene and additional phenotypic features.

Authors:  Michael Weinstein; Els Schollen; Gert Matthijs; Christine Neupert; Thierry Hennet; Claudia E Grubenmann; Christian G Frank; Markus Aebi; Joe T R Clarke; Anne Griffiths; Lorne Seargeant; Nicola Poplawski
Journal:  Am J Med Genet A       Date:  2005-07-15       Impact factor: 2.802

5.  New autosomal recessive lethal disorder with polycystic kidneys type Potter I, characteristic face, microcephaly, brachymelia, and congenital heart defects.

Authors:  G Gillessen-Kaesbach; P Meinecke; C Garrett; B C Padberg; H Rehder; E Passarge
Journal:  Am J Med Genet       Date:  1993-02-15

6.  Quality control of glycoproteins bearing truncated glycans in an ALG9-defective (CDG-IL) patient.

Authors:  Wendy Vleugels; Liesbeth Keldermans; Jaak Jaeken; Terry D Butters; Jean-Claude Michalski; Gert Matthijs; François Foulquier
Journal:  Glycobiology       Date:  2009-05-18       Impact factor: 4.313

7.  Paediatric age-dependent serum transferrin isoform distribution studied by HPLC.

Authors:  Torsten Arndt; Sven Stanzel; Adrian C Sewell
Journal:  Clin Lab       Date:  2007       Impact factor: 1.138

Review 8.  ALG8-CDG: novel patients and review of the literature.

Authors:  Michaela Höck; Karina Wegleiter; Elisabeth Ralser; Ursula Kiechl-Kohlendorfer; Sabine Scholl-Bürgi; Christine Fauth; Elisabeth Steichen; Karin Pichler; Dirk J Lefeber; Gert Matthjis; Liesbeth Keldermans; Kathrin Maurer; Johannes Zschocke; Daniela Karall
Journal:  Orphanet J Rare Dis       Date:  2015-06-12       Impact factor: 4.123

9.  Comprehensive gene panels provide advantages over clinical exome sequencing for Mendelian diseases.

Authors: 
Journal:  Genome Biol       Date:  2015-06-26       Impact factor: 13.583

10.  Diagnosis of ALG12-CDG by exome sequencing in a case of severe skeletal dysplasia.

Authors:  Chaya Murali; James T Lu; Mahim Jain; David S Liu; Ralph Lachman; Richard A Gibbs; Brendan H Lee; Daniel Cohn; Philippe M Campeau
Journal:  Mol Genet Metab Rep       Date:  2014
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  6 in total

Review 1.  What is new in CDG?

Authors:  Jaak Jaeken; Romain Péanne
Journal:  J Inherit Metab Dis       Date:  2017-05-08       Impact factor: 4.982

Review 2.  Liver involvement in congenital disorders of glycosylation (CDG). A systematic review of the literature.

Authors:  D Marques-da-Silva; V Dos Reis Ferreira; M Monticelli; P Janeiro; P A Videira; P Witters; J Jaeken; D Cassiman
Journal:  J Inherit Metab Dis       Date:  2017-01-20       Impact factor: 4.982

Review 3.  Cardiac complications of congenital disorders of glycosylation (CDG): a systematic review of the literature.

Authors:  D Marques-da-Silva; R Francisco; D Webster; V Dos Reis Ferreira; J Jaeken; T Pulinilkunnil
Journal:  J Inherit Metab Dis       Date:  2017-07-19       Impact factor: 4.982

4.  Classical Galactosaemia and CDG, the N-Glycosylation Interface. A Review.

Authors:  Ashwini Maratha; Hugh-Owen Colhoun; Ina Knerr; Karen P Coss; Peter Doran; Eileen P Treacy
Journal:  JIMD Rep       Date:  2016-08-09

5.  ALG9-CDG: New clinical case and review of the literature.

Authors:  Kellie Davis; Duncan Webster; Chris Smith; Sheryl Jackson; David Sinasac; Lorne Seargeant; Xing-Chang Wei; Patrick Ferreira; Julian Midgley; Yolanda Foster; Xueli Li; Miao He; Walla Al-Hertani
Journal:  Mol Genet Metab Rep       Date:  2017-09-06

6.  Liver Involvement in Congenital Disorders of Glycosylation: A Systematic Review.

Authors:  Rossella Colantuono; Elisa D'Acunto; Daniela Melis; Pietro Vajro; Hudson H Freeze; Claudia Mandato
Journal:  J Pediatr Gastroenterol Nutr       Date:  2021-10-01       Impact factor: 3.288

  6 in total

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