Dec 17, 2009

Neuropathy target esterase: an essential enzyme for neural development and axonal maintenance

The International Journal of Biochemistry & Cell Biology
Ping-An Chang, Yi-Jun Wu


Neuropathy target esterase (NTE) is an endoplasmic reticulum-anchored protein conserved across species. The N-terminal regulatory region of NTE contains three cyclic nucleotide binding domains while the C-terminal catalytic domain has a patatin domain. The NTE gene is expressed in mouse early at embryonic day 7 and its expression is maintained throughout embryonic development. NTE protein is mainly distributed in the nervous system with a pattern that is more restricted to large neurons in older animals. NTE regulates phospholipid metabolism and is known to be a phospholipase B. Knockout of NTE is embryonic lethal in mice, indicating that NTE is essential for embryonic survival. Neuronal specific NTE knockouts survive to adulthood, but show vacuolation and neuronal loss characteristic of neurodegenerative diseases. Recently, mutations in human NTE have been shown to cause a hereditary spastic paraplegia called NTE-related motor neuron disorder, suggesting a critical role for NTE in the nervous system.

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Mentioned in this Paper

Spastic Paraplegia 39, Autosomal Recessive
Biochemical Pathway
Cyclic Nucleotide Binding
Spastic Paraplegia, Hereditary
NTE, lymphocyte neuropathy target esterase
Abnormal Degeneration
Carboxy-Terminal Amino Acid
Entire Embryo
Malignant Neoplasm of Spinal Cord

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