PMID- 6410678
OWN - NLM
STAT- MEDLINE
DCOM- 19830923
LR  - 20190812
IS  - 0001-6322 (Print)
IS  - 0001-6322 (Linking)
VI  - 60
IP  - 1-2
DP  - 1983
TI  - New enzymatic findings in infantile neuroaxonal dystrophy.
PG  - 153-5
AB  - Two siblings with infantile neuroaxonal dystrophy (INAD) exhibited highly 
      increased activity of nonspecific esterase (resistant to physostigmine and 
      sensitive to organophosphates) topically correlating with the morphological 
      manifestations of the disease (spheroids), confined in those two particular cases 
      to the central nervous system (CNS). This is demonstrated for the first time. 
      NADH tetrazolium reductase was another enzyme noted with increased activity and 
      similar topography. The lesions were devoid of any marked activity of typical 
      mitochondrial (succinate and alpha-glycerolphosphate) dehydrogenases and 
      lysosomal (acid phosphatase and beta-glucuronidase) enzymes. The diagnostic 
      significance of monitoring non-specific esterase and NADH tetrazolium reductase 
      activities in the diagnosis of INAD is discussed in particular.
FAU - Elleder, M
AU  - Elleder M
FAU - Jirasek, A
AU  - Jirasek A
LA  - eng
PT  - Journal Article
PL  - Germany
TA  - Acta Neuropathol
JT  - Acta neuropathologica
JID - 0412041
RN  - EC 1.1.- (Glycerolphosphate Dehydrogenase)
RN  - EC 1.3.99.1 (Succinate Dehydrogenase)
RN  - EC 1.6.- (NADH Tetrazolium Reductase)
RN  - EC 3.1.- (Esterases)
RN  - EC 3.1.3.2 (Acid Phosphatase)
RN  - EC 3.2.1.31 (Glucuronidase)
SB  - IM
MH  - Acid Phosphatase/analysis
MH  - Brain/enzymology
MH  - Brain Diseases, Metabolic/*enzymology
MH  - Esterases/analysis
MH  - Glucuronidase/analysis
MH  - Glycerolphosphate Dehydrogenase/analysis
MH  - Humans
MH  - NADH Tetrazolium Reductase/analysis
MH  - Succinate Dehydrogenase/analysis
EDAT- 1983/01/01 00:00
MHDA- 1983/01/01 00:01
CRDT- 1983/01/01 00:00
PHST- 1983/01/01 00:00 [pubmed]
PHST- 1983/01/01 00:01 [medline]
PHST- 1983/01/01 00:00 [entrez]
AID - 10.1007/BF00685362 [doi]
PST - ppublish
SO  - Acta Neuropathol. 1983;60(1-2):153-5. doi: 10.1007/BF00685362.