Biomedical subjects
Publications and source records attributed to K Worley.
Primary immunodeficiencies are rare immune system disorders that present with recurrent and chronic infections of the head and neck. Otitis media, sinusitis, and upper respiratory tract infections develop frequently and present in early childhood. The purpose of this report is to classify and define the primary immunodeficiencies as well as to examine the clinical manifestations, evaluation, and treatment of these disorders.
Porin is the pore-forming protein involved in the movement of adenine nucleotides across the outer mitochondrial membrane (OMM). Hexokinase and glycerol kinase interact with porin on the outer surface of the OMM in a manner which provides these enzymes with preferred access to the ATP generated in the mitochondrion. We review recent evidence which permits refinement of our knowledge of these proteins and their interactions at the OMM. The involvement of this system in metabolic microcompartmentation is discussed, as well as possible pathological consequences of its disruption in malignancy and genetic deficiencies of hexokinase, glycerol kinase, and porin.