Most common cause of arsenic poisoning involves ARSENITE complexing stably with thiol groups of lipoic acid
>Coenzyme for: PDC E2 (dihydrolipoyl transacetylase), alpha-ketogluterate dehydrogenase, and branched-chain alpha-keto acid dehydrogenase.
-Usually caused from ground water, gold mining, rice
-Chronic consumption of arsenic is poisonous and even lethal.
-Symptoms: Headaches, confusion, diarrhea, and drowsiness; progression to vomiting, hematuria, cramping, hair loss, coma, and even death
*ARSENATE acts on glycolysis competing with phosphate; ARSENITE competes on TCA cycle by binding Lipoate
Subacute necrotizing encephalomyelopathy/ Leigh Syndrome
-Early-onset progressive neurodegenerative disorder with characteristic neuropathology consisting of focal, bilateral lesions in one or more ares of the CNS: brainstem, thalamus, basal ganglia, cerebellum, and spinal cord.
-Lesions: demyleination, gliosis, necrosis, spongiosis, capillary proliferation.
-Clinical symptoms depend on which areas of the CNS are involved.
-Due to deficiency of ANY ETC complex or substrate (PDC, PC)
Inheritance: mitochondrial, autosomal, or x-linked