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Article ; Online: An alternative approach to produce versatile retinal organoids with accelerated ganglion cell development.

Wagstaff, Ellie L / Ten Asbroek, Anneloor L M A / Ten Brink, Jacoline B / Jansonius, Nomdo M / Bergen, Arthur A B

2021 Volume 11, Issue 1, Page(s) 1101

Abstract: Genetically complex ocular neuropathies, such as glaucoma, are a major cause of visual impairment worldwide. There is a growing need to generate suitable human representative in vitro and in vivo models, as there is no effective treatment available once ... ...

Abstract	Genetically complex ocular neuropathies, such as glaucoma, are a major cause of visual impairment worldwide. There is a growing need to generate suitable human representative in vitro and in vivo models, as there is no effective treatment available once damage has occured. Retinal organoids are increasingly being used for experimental gene therapy, stem cell replacement therapy and small molecule therapy. There are multiple protocols for the development of retinal organoids available, however, one potential drawback of the current methods is that the organoids can take between 6 weeks and 12 months on average to develop and mature, depending on the specific cell type wanted. Here, we describe and characterise a protocol focused on the generation of retinal ganglion cells within an accelerated four week timeframe without any external small molecules or growth factors. Subsequent long term cultures yield fully differentiated organoids displaying all major retinal cell types. RPE, Horizontal, Amacrine and Photoreceptors cells were generated using external factors to maintain lamination.
MeSH term(s)	Cell Differentiation ; Cell Line ; Ganglia/cytology ; Humans ; Organoids/cytology ; Photoreceptor Cells/cytology ; Retina/cytology ; Retinal Ganglion Cells/cytology
Language	English
Publishing date	2021-01-13
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2615211-3
ISSN	2045-2322 ; 2045-2322
ISSN (online)	2045-2322
ISSN	2045-2322
DOI	10.1038/s41598-020-79651-x
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Article: Pseudoxanthoma elasticum: the end of the autosomal dominant segregation myth.

Bergen, Arthur A B

The Journal of investigative dermatology

2006 Volume 126, Issue 4, Page(s) 704–705

Abstract: Pseudoxanthoma elasticum (PXE) is a heritable connective-tissue disorder affecting the eye, skin, and vascular system. Recent publications show that PXE exclusively segregates in an autosomal recessive fashion. However, the lack of an internationally ... ...

Abstract	Pseudoxanthoma elasticum (PXE) is a heritable connective-tissue disorder affecting the eye, skin, and vascular system. Recent publications show that PXE exclusively segregates in an autosomal recessive fashion. However, the lack of an internationally accepted clinical "gold standard" for PXE, our incomplete knowledge of PXE etiology, and the incomplete nature of some molecular, clinical, and environmental studies warrant further investigation.
MeSH term(s)	Genes, Dominant ; Genes, Recessive ; Humans ; Pseudoxanthoma Elasticum/genetics
Language	English
Publishing date	2006-04
Publishing country	United States
Document type	Comment ; Journal Article
ZDB-ID	80136-7
ISSN	1523-1747 ; 0022-202X
ISSN (online)	1523-1747
ISSN	0022-202X
DOI	10.1038/sj.jid.5700129
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Article ; Online: Genetic deletion of Abcc6 disturbs cholesterol homeostasis in mice.

Ibold, Bettina / Tiemann, Janina / Faust, Isabel / Ceglarek, Uta / Dittrich, Julia / Gorgels, Theo G M F / Bergen, Arthur A B / Vanakker, Olivier / Van Gils, Matthias / Knabbe, Cornelius / Hendig, Doris

Scientific reports

2021 Volume 11, Issue 1, Page(s) 2137

Abstract: Genetic studies link adenosine triphosphate-binding cassette transporter C6 (ABCC6) mutations to pseudoxanthoma elasticum (PXE). ABCC6 sequence variations are correlated with altered HDL cholesterol levels and an elevated risk of coronary artery diseases. ...

Abstract	Genetic studies link adenosine triphosphate-binding cassette transporter C6 (ABCC6) mutations to pseudoxanthoma elasticum (PXE). ABCC6 sequence variations are correlated with altered HDL cholesterol levels and an elevated risk of coronary artery diseases. However, the role of ABCC6 in cholesterol homeostasis is not widely known. Here, we report reduced serum cholesterol and phytosterol levels in Abcc6-deficient mice, indicating an impaired sterol absorption. Ratios of cholesterol precursors to cholesterol were increased, confirmed by upregulation of hepatic 3-hydroxy-3-methylglutaryl coenzyme A reductase (Hmgcr) expression, suggesting activation of cholesterol biosynthesis in Abcc6
MeSH term(s)	Adult ; Animals ; Cholesterol/blood ; Cholesterol/metabolism ; Cholesterol, LDL/blood ; Cholesterol, LDL/metabolism ; Female ; Gene Deletion ; Gene Expression Profiling/methods ; Homeostasis/genetics ; Humans ; Lipids/blood ; Liver/metabolism ; Male ; Mice, 129 Strain ; Mice, Inbred C57BL ; Mice, Knockout ; Middle Aged ; Multidrug Resistance-Associated Proteins/deficiency ; Multidrug Resistance-Associated Proteins/genetics ; Proprotein Convertase 9/blood ; Proprotein Convertase 9/genetics ; Proprotein Convertase 9/metabolism ; Receptors, LDL/genetics ; Receptors, LDL/metabolism ; Mice
Chemical Substances	Abcc6 protein, mouse ; Cholesterol, LDL ; Lipids ; Multidrug Resistance-Associated Proteins ; Receptors, LDL ; Cholesterol (97C5T2UQ7J) ; Proprotein Convertase 9 (EC 3.4.21.-)
Language	English
Publishing date	2021-01-22
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2615211-3
ISSN	2045-2322 ; 2045-2322
ISSN (online)	2045-2322
ISSN	2045-2322
DOI	10.1038/s41598-021-81573-1
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Article ; Online: Identification of a Novel ZNF469 Mutation in a Pakistani Family With Brittle Cornea Syndrome.

Micheal, Shazia / Siddiqui, Sorath Noorani / Zafar, Saemah Nuzhat / Gabriëla Niewold, Ilse Therésia / Khan, Muhammad Imran / Bergen, Arthur A B

Cornea

2019 Volume 38, Issue 6, Page(s) 718–722

Abstract: Purpose: Brittle cornea syndrome (BCS) is a rare recessive disorder affecting connective tissues, most prominently in the eye. Pathogenic mutations causing BCS have been identified in PRDM5 and ZNF469 genes. This study investigates the genetic cause of ... ...

Abstract	Purpose: Brittle cornea syndrome (BCS) is a rare recessive disorder affecting connective tissues, most prominently in the eye. Pathogenic mutations causing BCS have been identified in PRDM5 and ZNF469 genes. This study investigates the genetic cause of BCS in a large, consanguineous Pakistani family with 4 affected and 3 unaffected individuals. Methods: The coding region and exon-intron splice junctions of PRDM5 and ZNF469 genes were amplified by polymerase chain reaction, and bidirectional Sanger sequencing was performed to find the pathogenic change responsible for causing the disease in the family. Results: A novel homozygous duplication c.9831dupC (p.Arg3278GlnfsX197) in the ZNF469 gene was identified, which was found to be co-segregating with the disease in the family. Conclusions: This is the first report of a ZNF469 homozygous mutation causing a BCS phenotype in a consanguineous Pakistani family. Our data extend the mutation spectrum of ZNF469 variants implicated in BCS.
MeSH term(s)	Child ; Child, Preschool ; Eye Abnormalities/genetics ; Female ; Humans ; Joint Instability/congenital ; Joint Instability/genetics ; Male ; Mutation ; Pakistan ; Skin Abnormalities/genetics ; Transcription Factors/genetics
Chemical Substances	Transcription Factors ; ZNF469 protein, human
Language	English
Publishing date	2019-03-13
Publishing country	United States
Document type	Journal Article
ZDB-ID	604826-2
ISSN	1536-4798 ; 0277-3740
ISSN (online)	1536-4798
ISSN	0277-3740
DOI	10.1097/ICO.0000000000001828
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Article ; Online: Abcc6 deficiency in mice leads to altered ABC transporter gene expression in metabolic active tissues.

Ibold, Bettina / Faust, Isabel / Tiemann, Janina / Gorgels, Theo G M F / Bergen, Arthur A B / Knabbe, Cornelius / Hendig, Doris

Lipids in health and disease

2019 Volume 18, Issue 1, Page(s) 2

Abstract: Background: ATP-binding cassette (ABC) transporters are involved in a huge range of physiological processes. Mutations in the ABCC6 gene cause pseudoxanthoma elasticum, a metabolic disease with progressive soft tissue calcification.: Methods: The aim ...

Abstract	Background: ATP-binding cassette (ABC) transporters are involved in a huge range of physiological processes. Mutations in the ABCC6 gene cause pseudoxanthoma elasticum, a metabolic disease with progressive soft tissue calcification. Methods: The aim of the present study was to analyze gene expression levels of selected ABC transporters associated with cholesterol homeostasis in metabolic active tissues, such as the liver, kidney and white adipose tissue (WAT) of Abcc6 Results: The strongest regulation of ABC transporter genes was observed in the liver tissue of six-month-old Abcc6 Conclusions: These data support previous findings that Abcc6 deficiency leads to an altered gene expression of other ABC transporters depending on the status of disease progression. The increased expression of fatty acid, bile salt and cholesterol/sterol transporters may be linked to an altered cholesterol and lipoprotein metabolism due to a loss of Abcc6 function.
MeSH term(s)	ATP Binding Cassette Transporter, Subfamily B/genetics ; ATP Binding Cassette Transporter, Subfamily B/metabolism ; ATP Binding Cassette Transporter, Subfamily B, Member 11/genetics ; ATP Binding Cassette Transporter, Subfamily B, Member 11/metabolism ; ATP Binding Cassette Transporter, Subfamily D/genetics ; ATP Binding Cassette Transporter, Subfamily D/metabolism ; ATP Binding Cassette Transporter, Subfamily G, Member 1/genetics ; ATP Binding Cassette Transporter, Subfamily G, Member 1/metabolism ; ATP Binding Cassette Transporter, Subfamily G, Member 5/genetics ; ATP Binding Cassette Transporter, Subfamily G, Member 5/metabolism ; ATP Binding Cassette Transporter, Subfamily G, Member 8/genetics ; ATP Binding Cassette Transporter, Subfamily G, Member 8/metabolism ; ATP-Binding Cassette Transporters/deficiency ; ATP-Binding Cassette Transporters/genetics ; ATP-Binding Cassette Transporters/metabolism ; Adipose Tissue, White/metabolism ; Adipose Tissue, White/pathology ; Animals ; Bile Acids and Salts/metabolism ; Biological Transport ; Cholesterol/metabolism ; Disease Models, Animal ; Disease Progression ; Female ; Gene Expression Regulation ; Kidney/metabolism ; Kidney/pathology ; Lipoproteins/genetics ; Lipoproteins/metabolism ; Liver/metabolism ; Liver/pathology ; Male ; Mice ; Mice, Knockout ; Pseudoxanthoma Elasticum/genetics ; Pseudoxanthoma Elasticum/metabolism ; Pseudoxanthoma Elasticum/pathology
Chemical Substances	ABCD2 protein, mouse ; ABCG1 protein, mouse ; ABCG5 protein, mouse ; ABCG8 protein, mouse ; ATP Binding Cassette Transporter, Subfamily B ; ATP Binding Cassette Transporter, Subfamily B, Member 11 ; ATP Binding Cassette Transporter, Subfamily D ; ATP Binding Cassette Transporter, Subfamily G, Member 1 ; ATP Binding Cassette Transporter, Subfamily G, Member 5 ; ATP Binding Cassette Transporter, Subfamily G, Member 8 ; ATP-Binding Cassette Transporters ; Abca3 protein, mouse ; Abcb11 protein, mouse ; Abcc6 protein, mouse ; Bile Acids and Salts ; Lipoproteins ; Cholesterol (97C5T2UQ7J) ; Abcb1b protein, mouse (EC 7.6.2.2)
Language	English
Publishing date	2019-01-05
Publishing country	England
Document type	Journal Article
ISSN	1476-511X
ISSN (online)	1476-511X
DOI	10.1186/s12944-018-0943-x
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Article ; Online: Human ciliary epithelia do express genes with retinal progenitor cell characteristics in vivo.

Janssen, Sarah F / Bennis, Anna / Heine, Vivi M / Bergen, Arthur A B

Experimental eye research

2014 Volume 121, Page(s) 41

MeSH term(s)	Animals ; Ciliary Body/growth & development ; Regeneration/physiology ; Vertebrates
Language	English
Publishing date	2014-04
Publishing country	England
Document type	Comment ; Letter
ZDB-ID	80122-7
ISSN	1096-0007 ; 0014-4835
ISSN (online)	1096-0007
ISSN	0014-4835
DOI	10.1016/j.exer.2014.01.020
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Article: Delineation of Novel Autosomal Recessive Mutation in GJA3 and Autosomal Dominant Mutations in GJA8 in Pakistani Congenital Cataract Families.

Micheal, Shazia / Niewold, Ilse Therésia Gabriëla / Siddiqui, Sorath Noorani / Zafar, Saemah Nuzhat / Khan, Muhammad Imran / Bergen, Arthur A B

Genes

2018 Volume 9, Issue 2

Abstract: Congenital cataract is a clinically and genetically heterogeneous disease. The present study was undertaken to find the genetic cause of congenital cataract families. DNA samples of a large consanguineous Pakistani family were genotyped with a high ... ...

Abstract	Congenital cataract is a clinically and genetically heterogeneous disease. The present study was undertaken to find the genetic cause of congenital cataract families. DNA samples of a large consanguineous Pakistani family were genotyped with a high resolution single nucleotide polymorphism Illumina microarray. Homozygosity mapping identified a homozygous region of 4.4 Mb encompassing the gene
Language	English
Publishing date	2018-02-20
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2527218-4
ISSN	2073-4425
ISSN	2073-4425
DOI	10.3390/genes9020112
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Article ; Online: Novel mutations in the PITX2 gene in Pakistani and Mexican families with Axenfeld-Rieger syndrome.

Lo Faro, Valeria / Siddiqui, Sorath N / Khan, Muhammad I / Villanueva-Mendoza, Cristina / Cortés-González, Vianney / Jansonius, Nomdo / Bergen, Arthur A B / Micheal, Shazia

Molecular genetics & genomic medicine

2020 Volume 8, Issue 7, Page(s) e1215

Abstract: Purpose: Axenfeld-Rieger syndrome (ARS) is a rare autosomal dominant disorder that affects the anterior segment of the eye. The aim of this study was to examine the PITX2 gene to identify possible novel mutations in Pakistani and Mexican families ... ...

Abstract	Purpose: Axenfeld-Rieger syndrome (ARS) is a rare autosomal dominant disorder that affects the anterior segment of the eye. The aim of this study was to examine the PITX2 gene to identify possible novel mutations in Pakistani and Mexican families affected by the ARS phenotype. Methods: Three unrelated probands with a diagnosis of ARS were recruited for this study. Genomic DNA was isolated from the peripheral blood of the probands and their family members. Polymerase chain reaction and Sanger sequencing were used for the analysis of coding exons and the flanking intronic regions of the PITX2 gene. Bioinformatics tools and database (VarSome, Provean, and MutationTaster, SIFT, PolyPhen-2, and HOPE) were evaluated to explore missense variants. Results: We identified novel heterozygous variations in the PITX2 gene that segregated with the ARS phenotype within the families. The variant NM_153426.2(PITX2):c.226G > T or p.(Ala76Ser) and the mutation NM_153426.2(PITX2):c.455G > A or p.(Cys152Tyr) were identified in two Pakistani pedigrees, and the mutation NM_153426.2(PITX2):c.242_265del or p.(Lys81_Gln88del), segregated in a Mexican family. Conclusion: Our study extends the spectrum of PITX2 mutations in individuals with ARS, enabling an improved diagnosis of this rare but serious syndrome.
MeSH term(s)	Adolescent ; Anterior Eye Segment/abnormalities ; Anterior Eye Segment/pathology ; Child ; Eye Abnormalities/genetics ; Eye Abnormalities/pathology ; Eye Diseases, Hereditary/genetics ; Eye Diseases, Hereditary/pathology ; Female ; Heterozygote ; Homeodomain Proteins/genetics ; Humans ; Male ; Mutation ; Pedigree ; Transcription Factors/genetics ; Homeobox Protein PITX2
Chemical Substances	Homeodomain Proteins ; Transcription Factors
Language	English
Publishing date	2020-05-13
Publishing country	United States
Document type	Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2734884-2
ISSN	2324-9269 ; 2324-9269
ISSN (online)	2324-9269
ISSN	2324-9269
DOI	10.1002/mgg3.1215
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Article ; Online: A review of treatment modalities in gyrate atrophy of the choroid and retina (GACR).

Balfoort, Berith M / Buijs, Mark J N / Ten Asbroek, Anneloor L M A / Bergen, Arthur A B / Boon, Camiel J F / Ferreira, Elise A / Houtkooper, Riekelt H / Wagenmakers, Margreet A E M / Wanders, Ronald J A / Waterham, Hans R / Timmer, Corrie / van Karnebeek, Clara D / Brands, Marion M

Molecular genetics and metabolism

2021 Volume 134, Issue 1-2, Page(s) 96–116

Abstract: Gyrate atrophy of the choroid and retina (GACR) is a rare inborn error of amino acid metabolism caused by bi-allelic variations in OAT. GACR is characterised by vision decline in early life eventually leading to complete blindness, and high plasma ... ...

Abstract	Gyrate atrophy of the choroid and retina (GACR) is a rare inborn error of amino acid metabolism caused by bi-allelic variations in OAT. GACR is characterised by vision decline in early life eventually leading to complete blindness, and high plasma ornithine levels. There is no curative treatment for GACR, although several therapeutic modalities aim to slow progression of the disease by targeting different steps within the ornithine pathway. No international treatment protocol is available. We systematically collected all international literature on therapeutic interventions in GACR to provide an overview of published treatment effects. Methods: Following the PRISMA guidelines, we conducted a systematic review of the English literature until December 22nd 2020. PubMed and Embase databases were searched for studies related to therapeutic interventions in patients with GACR. Results: A total of 33 studies (n = 107 patients) met the inclusion criteria. Most studies were designed as case reports (n = 27) or case series (n = 4). No randomised controlled trials or large cohort studies were found. Treatments applied were protein-restricted diets, pyridoxine supplementation, creatine or creatine precursor supplementation, l-lysine supplementation, and proline supplementation. Protein-restricted diets lowered ornithine levels ranging from 16.0-91.2%. Pyridoxine responsiveness was reported in 30% of included mutations. Lysine supplementation decreased ornithine levels with 21-34%. Quality assessment showed low to moderate quality of the articles. Conclusions: Based primarily on case reports ornithine levels can be reduced by using a protein restricted diet, pyridoxine supplementation (variation-dependent) and/or lysine supplementation. The lack of pre-defined clinical outcome measures and structural follow-up in all included studies impeded conclusions on clinical effectiveness. Future research should be aimed at 1) Unravelling the OAT biochemical pathway to identify other possible pathologic metabolites besides ornithine, 2) Pre-defining GACR specific clinical outcome measures, and 3) Establishing an international historical cohort.
MeSH term(s)	Choroid/drug effects ; Choroid/pathology ; Gyrate Atrophy/drug therapy ; Humans ; Metabolism, Inborn Errors/drug therapy ; Mutation ; Retina/drug effects ; Retina/pathology
Language	English
Publishing date	2021-07-26
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	1418518-0
ISSN	1096-7206 ; 1096-7192
ISSN (online)	1096-7206
ISSN	1096-7192
DOI	10.1016/j.ymgme.2021.07.010
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Article ; Online: The SERPING1 gene and age-related macular degeneration.

Klaver, Caroline C W / Bergen, Arthur A B

Lancet (London, England)

2008 Volume 372, Issue 9652, Page(s) 1788–1789

MeSH term(s)	Complement C1 Inactivator Proteins/genetics ; Complement C1 Inhibitor Protein ; Humans ; Macular Degeneration/genetics ; Polymorphism, Single Nucleotide/genetics ; Risk Factors
Chemical Substances	Complement C1 Inactivator Proteins ; Complement C1 Inhibitor Protein ; SERPING1 protein, human
Language	English
Publishing date	2008-11-22
Publishing country	England
Document type	Journal Article ; Comment
ZDB-ID	3306-6
ISSN	1474-547X ; 0023-7507 ; 0140-6736
ISSN (online)	1474-547X
ISSN	0023-7507 ; 0140-6736
DOI	10.1016/S0140-6736(08)61349-5
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