PMID- 10540014 OWN - NLM STAT- MEDLINE DCOM- 19991202 LR - 20190724 IS - 0022-510X (Print) IS - 0022-510X (Linking) VI - 169 IP - 1-2 DP - 1999 Oct 31 TI - A receptor for advanced glycosylation endproducts (AGEs) is colocalized with neurofilament-bound AGEs and SOD1 in motoneurons of ALS: immunohistochemical study. PG - 87-92 AB - Neurofilament (NF)-bound AGEs colocalize immunochemically with SOD1 in the motoneurons of patients with ALS. Among three types of AGE receptors reported in the human brain, AGE-R1 (oligosaccharyltransferase family) and AGE-R2 (substrate of protein kinase C) have been found in neurons, while AGE-R3 is restricted to glia. The present study investigates which of these receptors may be responsible for binding AGEs in the NF conglomerates of motoneurons. Immunostaining of paraffin sections from eight ALS patients (five sporadic and three familial) and three control cases was performed with antibodies directed against R1 and R2, in parallel with those against AGEs and SOD1. The sites of AGE-R1 immunoreactivity (IR) in motoneurons were in conformity to those of NF-associated AGE and SOD1 IRs. By contrast, the IR of R2 was negative in NF conglomerates. Negative R2 IR for NF conglomerates was outlined by surrounding coarse R2 immunopositive granules in the perikaryon. No IR for R1 or R2 was found in hyaline or Bunina inclusions. There was no extraneuronal expression of IR for AGE-R1 or AGEs in microglia or astroglia around the NF accumulation. The colocalization of AGE, AGE-R1, and SOD1 at NF conglomerates in motoneurons supports the notion that AGE-mediated oxidative stress and protein aggregation may be implicated in NF conglomeration and ALS pathogenesis. FAU - Chou, S M AU - Chou SM AD - Norris ALS/MDA Research Center, California Pacific Medical Center, San Francisco, CA 94116, USA. FAU - Han, C Y AU - Han CY FAU - Wang, H S AU - Wang HS FAU - Vlassara, H AU - Vlassara H FAU - Bucala, R AU - Bucala R LA - eng PT - Journal Article PL - Netherlands TA - J Neurol Sci JT - Journal of the neurological sciences JID - 0375403 RN - 0 (Receptor for Advanced Glycation End Products) RN - 0 (Receptors, Immunologic) RN - 0 (SOD1 protein, human) RN - EC 1.15.1.1 (Superoxide Dismutase) RN - EC 1.15.1.1 (Superoxide Dismutase-1) SB - IM MH - *Amyotrophic Lateral Sclerosis MH - Humans MH - Immunohistochemistry MH - Motor Neurons/*chemistry MH - Receptor for Advanced Glycation End Products MH - Receptors, Immunologic/*analysis MH - Superoxide Dismutase/*analysis MH - Superoxide Dismutase-1 EDAT- 1999/12/14 00:00 MHDA- 1999/12/14 00:01 CRDT- 1999/12/14 00:00 PHST- 1999/12/14 00:00 [pubmed] PHST- 1999/12/14 00:01 [medline] PHST- 1999/12/14 00:00 [entrez] AID - S0022-510X(99)00222-1 [pii] AID - 10.1016/s0022-510x(99)00222-1 [doi] PST - ppublish SO - J Neurol Sci. 1999 Oct 31;169(1-2):87-92. doi: 10.1016/s0022-510x(99)00222-1.