Acronym for Lethal Congenital Contractures Syndrome

Forms of arthrogryposis (see this term) that are usually lethal at birth or shortly thereafter.

They are:

-        LCCS 1 [MIM 253 510], GLE1 gene (9q34)

-        LCCS 2 [MIM 607 598], ERBB3 gene

-        LCCS 3 [MIM 611 369], PIP5K1C gene

-        LCCS 4 [MIM 614 915], DNM2 gene

-        LCCS 5 [MIM 615 368], DNMZ gene

-        LCCS 6 [MIM 616 248], ZBTB4Z gene

-        LCCS 7 [MIM 616 826], CNTAP1 (17q21.2) gene also called Boylan-Dew-Greco syndrome or arthrogryposis with demyelinating neuropathy

-        LCCS 8 [MIM 616 827], ADCY6 (12q13) gene

-        LCCS 9 [MIM 616 503], ADGRG6 gene

-        LCCS 10 [MIM 670 022], NEK9 gene

-        LCCS 11 [MIM 617 194], GLDN gene

Updated February 2019