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Meier-Gorlin syndrome

Updated: Wikipedia source

Meier-Gorlin syndrome

Meier-Gorlin syndrome, also known as ear-patella-short stature syndrome is a rare autosomal recessive genetic disorder, which is mainly characterized by pre- and postnatal growth deficiency, patellar aplasia or hypoplasia, and underdevelopment of both ears. Patients have characteristic facial signs, such as small mouth with full lips, receding jaw, hooked nose, and small ears with abnormal shape. Meier-Gorlin syndrome is considered to be one of the forms of microcephalic primordial dwarfism. Fewer than 150 cases had been recorded as of 2024.

Infobox

Other names
Ear-patella-short stature syndrome, MGORS
Symptoms
IUGR, short stature, patellar hypoplasia or aplasia, microtia, micromastia, pulmonary emphysema.
Types
8
Causes
Mutations in the genes ORC1, ORC4, ORC6, CDT1, CDC6, CDC45L, MCM5, GMNN.
Treatment
Symptomatic
Frequency
1-9:1 000 000

Tables

· Cause
MGORS1
MGORS1
Type
MGORS1
Gene
ORC1
OMIM
224690
Inheritance
Autosomal recessive
MGORS2
MGORS2
Type
MGORS2
Gene
ORC4
OMIM
613800
Inheritance
Autosomal recessive
MGORS3
MGORS3
Type
MGORS3
Gene
ORC6
OMIM
613803
Inheritance
Autosomal recessive
MGORS4
MGORS4
Type
MGORS4
Gene
CDT1
OMIM
613804
Inheritance
Autosomal recessive
MGORS5
MGORS5
Type
MGORS5
Gene
CDC6
OMIM
613805
Inheritance
Autosomal recessive
MGORS6
MGORS6
Type
MGORS6
Gene
GMNN
OMIM
616835
Inheritance
Autosomal dominant
MGORS7
MGORS7
Type
MGORS7
Gene
CDC45L
OMIM
617063
Inheritance
Autosomal recessive
MGORS8
MGORS8
Type
MGORS8
Gene
MCM5
OMIM
617564
Inheritance
Autosomal recessive
Type
Gene
OMIM
Inheritance
MGORS1
ORC1
224690
Autosomal recessive
MGORS2
ORC4
613800
Autosomal recessive
MGORS3
ORC6
613803
Autosomal recessive
MGORS4
CDT1
613804
Autosomal recessive
MGORS5
CDC6
613805
Autosomal recessive
MGORS6
GMNN
616835
Autosomal dominant
MGORS7
CDC45L
617063
Autosomal recessive
MGORS8
MCM5
617564
Autosomal recessive

References

  1. American Journal of Medical Genetics Part A
    https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.a.30899
  2. www.orpha.net
    http://www.orpha.net/en/disease/detail/2554
  3. American Journal of Medical Genetics Part A
    https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.a.35681
  4. Molecular Genetics and Genomics
    https://doi.org/10.1007%2Fs00438-015-1110-y
  5. Orphanet Journal of Rare Diseases
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11715027
  6. Orphanet Journal of Rare Diseases
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4574002
  7. www.orpha.net
    http://www.orpha.net/en/disease/sign/2554
  8. European Journal of Human Genetics
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3355263
  9. The American Journal of Human Genetics
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5005452
  10. BMC Pregnancy and Childbirth
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8130261
  11. www.omim.org
    https://www.omim.org/entry/224690?search=meier-gorlin%20syndrome&highlight=%22meier%20gorlin%22,(syndrome%7Csyndromic),meiergorlin
  12. www.cancer.gov
    https://www.cancer.gov/publications/dictionaries/cancer-terms/def/genetic-heterogeneity
  13. www.omim.org
    https://www.omim.org/entry/616835
  14. Journal of Biological Chemistry
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2843193
  15. meshb.nlm.nih.gov
    https://meshb.nlm.nih.gov/record/ui?name=Origin+Recognition+Complex
  16. DNA Repair
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6764890
  17. PLOS Genetics
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6742236
  18. European Journal of Human Genetics
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10400559
  19. The American Journal of Human Genetics
    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4678788
  20. Helvetica Paediatrica Acta
    https://search.worldcat.org/issn/0018-022X
  21. Birth Defects Original Article Series
    https://search.worldcat.org/issn/0547-6844
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