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. 2022 Jan 24;13:780930. doi: 10.3389/fgene.2022.780930

TABLE 3.

Sequence variations found in SMMCI (not HPE) and its main clinical findings.

Gene Nucleotide variation Main clinical manifestations Reference
SHH c.331A > T p.I111F SMMCI, choanal stenosis Nanni et al. (2001)
SHH c.331A > T p.I111F SMMCI, choanal stenosis, slow learner
SHH c.995T > C p.V332A SMMCI, choanal stenosis, hypotelorism, microcephaly, patent ductus arteriosus, premaxillary region dysplasia Dubourg et al. (2004)
SHH c.995T > C p.V332A SMMCI, cleft palate, hypotelorism, short stature, corpus callosum dysplasia, colpocephaly
SHH c.420C > G p.H140Q SMMCI, hypotelorism, microcephaly, neurohypophyseal tumor Ribeiro and Richieri-Costa (2005)
SIX3 c.686C > T p.P229L SMMCI, hypotelorism El-Jaick et al. (2007)
SIX3 c.109G > T p.G37C SMMCI, cleft lip/palate, choanal atresia, ptosis, coloboma, microcephaly, short stature, mild intellectual disability, ventricular septal defect Poelmans et al. (2015)
TGIF1 c.83C > G p.S28C SMMCI, congenital nasal pyriform aperture stenosis, hypotelorism, microcephaly, growth retardation, corpus callosum dysplasia Gripp et al. (2000)
COL4A2 c.3896G > A p.G1299E SMMCI, congenital nasal pyriform aperture stenosis, microcephaly, growth retardation, schizencephaly, dermoid cyst Gazdagh et al. (2017)
COL4A2 c.3896G > A p.G1299E SMMCI, delayed speech, dermoid cyst
DISP1 c.4049delC p.S1350fs SMMCI, choana stenosis, coloboma of iris and retina, microcephaly, growth hormone deficiency, growth retardation, corpus callosum dysplasia, delayed speech, epilepsy, central diabetes insipidus Garcia Rodriguez et al. (2019)
ZIC2 c.80C > T p.A27V
PTCH1 c.109G > T p.G37W
SIX3 c.514G > A p.A172T
ASLX1 c.583G > A p.A195T
SMO c.1265G > A p.G422E SMMCI, congenital nasal pyriform aperture stenosis Zatonski et al. (2020)
PLD2 c.956delA p.Q319fs
P2RY13 c.615G > A p.W205*