The X-axis shows the frequency of each malformation in each vertebra and
Y-axis shows the vertebral distribution in the spine. The vertebral
malformations in the non-TACS group (N=298) were normally distributed. There
were 58 TACS patients in total in three worldwide cohorts (Cohort 1 from PUMCH
in China, N=33; Cohort 2 from the multiple centers in Japan, N=15; Cohort 3 from
multiple centers in the USA, N=10). The
Tbx6−/mh engineered mice [N=52] exhibited
a distinct phenotype, namely, a defect of formation involving the lower part of
the spine. In addition, malformations in the upper and middle thoracic spine
were significantly less involved (P
=7.3×10−5 and 3.0×10−6,
respectively) in the TACS group than in the non-TACS group.
Abbreviation: CS, congenital scoliosis; TACS,
TBX6-associated CS; Non-TACS,
non-TBX6-associated CS; mh, mild-hypomorphic;
C, cervical vertebra; T, thoracic vertebra; L, lumbar vertebra; S, sacral
vertebra.