Literature DB >> 28742265

Congenital disorders of glycosylation: The Saudi experience.

Sarah Alsubhi1, Amal Alhashem2, Eissa Faqeih3, Majid Alfadhel4, Abdullah Alfaifi4, Waleed Altuwaijri5, Saud Alsahli4, Hesham Aldhalaan6, Fowzan S Alkuraya7,8, Khalid Hundallah1, Adel Mahmoud3, Ali Alasmari3, Fuad Al Mutairi4, Hanem Abduraouf2, Layan AlRasheed2, Saad Alshahwan1, Brahim Tabarki1.   

Abstract

We retrospectively reviewed Saudi patients who had a congenital disorder of glycosylation (CDG). Twenty-seven Saudi patients (14 males, 13 females) from 13 unrelated families were identified. Based on molecular studies, the 27 CDG patients were classified into different subtypes: ALG9-CDG (8 patients, 29.5%), ALG3-CDG (7 patients, 26%), COG6-CDG (7 patients, 26%), MGAT2-CDG (3 patients, 11%), SLC35A2-CDG (1 patient), and PMM2-CDG (1 patient). All the patients had homozygous gene mutations. The combined carrier frequency of CDG for the encountered founder mutations in the Saudi population is 11.5 per 10,000, which translates to a minimum disease burden of 14 patients per 1,000,000. Our study provides comprehensive epidemiologic information and prevalence figures for each of these CDG in a large cohort of congenital disorder of glycosylation patients.
© 2017 Wiley Periodicals, Inc.

Entities:  

Keywords:  ALG9; CDG; Congenital disorder(s) of glycosylation; severe phenotype; skeletal dysplasia

Mesh:

Substances:

Year:  2017        PMID: 28742265     DOI: 10.1002/ajmg.a.38358

Source DB:  PubMed          Journal:  Am J Med Genet A        ISSN: 1552-4825            Impact factor:   2.802


  12 in total

1.  ALG3-CDG: lethal phenotype and novel variants in Chinese siblings.

Authors:  Yue Bian; Chong Qiao; ShuGuang Zheng; Hao Qiu; Huan Li; ZhiTao Zhang; ShaoWei Yin; HongKun Jiang; Jesse Li-Ling; CaiXia Liu; Yuan Lyu
Journal:  J Hum Genet       Date:  2020-07-12       Impact factor: 3.172

2.  Secondary Hemophagocytic Syndrome Associated with COG6 Gene Defect: Report and Review.

Authors:  Nouf Althonaian; Abdulrahman Alsultan; Eva Morava; Majid Alfadhel
Journal:  JIMD Rep       Date:  2018-02-15

3.  Successful treatment of intractable epilepsy with ketogenic diet therapy in twins with ALG3-CDG.

Authors:  C Paketci; P Edem; S Hiz; E Sonmezler; D Soydemir; G Sarikaya Uzan; Y Oktay; E O'Heir; S Beltran; S Laurie; A Töpf; H Lochmuller; R Horvath; U Yis
Journal:  Brain Dev       Date:  2020-05-07       Impact factor: 1.961

4.  Expanding the phenotype, genotype and biochemical knowledge of ALG3-CDG.

Authors:  Hind Alsharhan; Bobby G Ng; Earnest James Paul Daniel; Jennifer Friedman; Eniko K Pivnick; Amal Al-Hashem; Eissa Ali Faqeih; Pengfei Liu; Nicole M Engelhardt; Kierstin N Keller; Jie Chen; Pamela A Mazzeo; Jill A Rosenfeld; Michael J Bamshad; Deborah A Nickerson; Kimiyo M Raymond; Hudson H Freeze; Miao He; Andrew C Edmondson; Christina Lam
Journal:  J Inherit Metab Dis       Date:  2021-03-01       Impact factor: 4.750

5.  Congenital disorders of glycosylation: Prevalence, incidence and mutational spectrum in the Polish population.

Authors:  Patryk Lipiński; Anna Bogdańska; Anna Tylki-Szymańska
Journal:  Mol Genet Metab Rep       Date:  2021-02-11

6.  Immune dysfunction in MGAT2-CDG: A clinical report and review of the literature.

Authors:  Sheri A Poskanzer; Matthew J Schultz; Coleman T Turgeon; Noemi Vidal-Folch; Kris Liedtke; Devin Oglesbee; Dimitar K Gavrilov; Silvia Tortorelli; Dietrich Matern; Piero Rinaldo; James T Bennett; Jenny M Thies; Irene J Chang; Anita E Beck; Kimiyo Raymond; Eric J Allenspach; Christina Lam
Journal:  Am J Med Genet A       Date:  2020-10-12       Impact factor: 2.802

Review 7.  ALG3-CDG: a patient with novel variants and review of the genetic and ophthalmic findings.

Authors:  Martina Farolfi; Anna Cechova; Nina Ondruskova; Jana Zidkova; Bohdan Kousal; Hana Hansikova; Tomas Honzik; Petra Liskova
Journal:  BMC Ophthalmol       Date:  2021-06-05       Impact factor: 2.209

8.  Mosaicism of the UDP-Galactose transporter SLC35A2 in a female causing a congenital disorder of glycosylation: a case report.

Authors:  Kristen Westenfield; Kyriakie Sarafoglou; Laura C Speltz; Elizabeth I Pierpont; Joan Steyermark; David Nascene; Matthew Bower; Mary Ella Pierpont
Journal:  BMC Med Genet       Date:  2018-06-15       Impact factor: 2.103

Review 9.  Maintaining order: COG complex controls Golgi trafficking, processing, and sorting.

Authors:  Jessica B Blackburn; Zinia D'Souza; Vladimir V Lupashin
Journal:  FEBS Lett       Date:  2019-08-16       Impact factor: 4.124

Review 10.  Genetic analysis and prenatal diagnosis in a Chinese with growth retardation, abnormal liver function, and microcephaly.

Authors:  Peiwei Zhao; Lei Zhang; Li Tan; Sukun Luo; Yufeng Huang; Hanming Peng; Jiangxia Cao; Xuelian He
Journal:  Mol Genet Genomic Med       Date:  2021-07-31       Impact factor: 2.183

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