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Description

Komponenti prodhohet natyrshm n trupin e njeriut prodhimi intrakorporal i L-carnitine ndodh n mli dhe veshka, dhe substanca grumbullohet kryesisht n muskujt e zemrs dhe muskujt skeletor

Discussion We report 3 probands from two unrelated families with rare BBOX1 variants, L-carnitine deficiency, and elevated levels of plasma -BB, indicative of a causative effect of the BBOX1 variants affecting the final stage of the L-carnitine biosynthetic pathway