Have a personal or library account? Click to login
Exploring pathogenesis, prevalence, and genetic associations in Chiari malformation type 1: a contemporary perspective Cover

Exploring pathogenesis, prevalence, and genetic associations in Chiari malformation type 1: a contemporary perspective

Open Access
|Sep 2024

Figures & Tables

List of candidate genes associated with CM 1

CountryGenesLocationFunction/effects of mutationRegion of mutation involvedAuthor
ChinaERFChromosome 19Multiple suture = 45.45% of the casesNM_006494.4(ERF): c.787C>T (p. Gln263Ter)Wang et al. [51]
Sagittal suture closure = 20%
RussiaMYBPC1Chromosome 12 (12q23.2)Affected individuals have less severe muscle contractions and restricted growth at the base of the skull.NM_002465.4(MYBPC1): c.517G>A (p. Asp173Asn)Musolf et al. [30]
COX20Chromosome 1 (1q43-44)Microcephaly, structural disorders of the brain and abnormalities of hands/feet.NM_198076.6(COX20): c.353A>G (p. Asn118Ser)
USACHD 3Chromosome 17Macrocephaly and development disorderNM_001005273.3(CHD3): c.50G>A (p. Cys17Tyr); c.70C>T (p. Arg24Trp) and c.74C>T (p. Ala25Val)Sadler et al. [31]
CHD8Chromosome 14Macrocephaly and autism spectrum disorderNM_001170629.2(CHD8): c.4414C>T (p. Arg1472Ter); c.4514 G>A (p. Trp1505Ter) and c.2907 + 1G>T
ItalyDKK1Chromosome 10Affects a pentapeptide motif (NAIKN) during binding with LRPs in WNT signaling.NM_012242.4 (DKK1): c.121G>A (p. Ala41Thr)Merello et al. [28]
SpainCOL7A1Chromosome 3Downregulated in articular cartilage and subchondral bone where osteoarthritis takes place.NM_000094.4(COL7A1): c.3605G>A (p. Arg1202His)Urbizu et al. [52]
SpainCOL6A5Chromosome 3Affects the bone mineral densityNM_001278298.2(COL6A5): c.6814G>T (p. Glu2272Ter)Urbizu et al. [52]
JapanFGFR2Chromosome 10Causes the Crouzon syndromeNM_000141.5(FGFR2): c.1205C>G (p. Cys342Trp) and c.1021A>G (p. Tyr281Cys)Fujisawa et al. [53]
USAGDF6Chromosome 8Controls proliferation and cellular differentiation in retina and bone formation. Affects BMP signaling.NM_001001557.4(GDF6): c.746C>A (p. Ala249Glu)Markunas et al. [16]
USAEPAS1Chromosome 2Spinal dysraphism and abnormal vertebral segmentationNM_001430.5(EPAS1): c.1589C>T (p. Ala530Val) and c.1588G>A (p. Tyr532Cys)Rosenblum et al. [54]
ItalyBMP1Chromosome 8Affects the metalloprotease domain which is important in osteogenesis.NM_001199.4(BMP1): c.941G>A (p. Arg314His)Merello et al. [28]
ItalyLRP4Chromosome 11Affects the LDL receptor class B repeat 7 which is part of third β-propeller domainNM_002334.4(LRP4): c.2552C>G (p. Thr851Arg)Merello et al. [28]
TurkishOLFML2AChromosome 9Role in development of brain structuresrs7874348Avşar et al. [55]
SLC4A9Chromosome 5Role in fluid secretion regulation (anion exchanger, especially Cl)rs6860077
DOI: https://doi.org/10.2478/abm-2024-0021 | Journal eISSN: 1875-855X | Journal ISSN: 1905-7415
Language: English
Page range: 148 - 156
Published on: Sep 20, 2024
Published by: Chulalongkorn University
In partnership with: Paradigm Publishing Services
Publication frequency: 6 issues per year

© 2024 Siti Nornazihah Mohd Rosdi, Suzuanhafizan Omar, Mazira Mohamad Ghazali, Ab Rahman Izaini Ghani, Abdul Aziz Mohamed Yusoff, published by Chulalongkorn University
This work is licensed under the Creative Commons Attribution 4.0 License.