LUZP deficiency affects neural tube closure during brain development.
LUZP is a leucine zipper-containing protein predominantly expressed in the brain. The functional significance of LUZP remains unknown. To explore the role of LUZP in brain development, a knockout mouse strain with a lacZ knock-in (Luzp-KO/lacZ-KI) has been established. LacZ reporter expression driven by the endogenous Luzp promoter was detected in the neuroepithelium and the cardiac tissue. Luzp(-/-) mice exhibited perinatal death, presumably due to the accompanied complex cardiovascular defects. Luzp(-/-) embryos displayed a cranial neural tube closure defect (NTD), with exposed brain tissues. Ectopic expression of Sonic-hedgehog, which is a protein known to be involved in neural tube closure, and elevated apoptosis were observed in the dorsal lateral neuroepithelium of the NTD Luzp(-/-) hindbrain. These findings assign a novel function of LUZP in the embryonic development of brain.
Hsu
Chia-Yi
CY
Institute of Neuroscience, School of Life Science, National Yang-Ming University, 155, Section 2, Linong Street, Taipei 11211, Taiwan.
Chang
Nan-Chi
NC
Lee
Maud Wan-Ying
MW
Lee
Kun-Hsiung
KH
Sun
Der-Shan
DS
Lai
Carol
C
Chang
Alice Chien
AC
D000818:Animals; D017209:Apoptosis; D001921:Brain / Q000166:cytology / Q000196:embryology* / Q000378:metabolism; D004268:DNA-Binding Proteins / Q000235:genetics / Q000502:physiology*; D053823:Hedgehog Proteins / Q000096:biosynthesis; D051379:Mice; D018345:Mice, Knockout; D054259:Neural Tube / Q000002:abnormalities* / Q000378:metabolism; D020411:Oligonucleotide Array Sequence Analysis; D012249:Rhombencephalon / Q000166:cytology / Q000196:embryology / Q000378:metabolism
D004268:DNA-Binding Proteins; D053823:Hedgehog Proteins; C103593:Luzp1 protein, mouse; C465570:Shh protein, mouse