+ Site Statistics
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Translate
+ Recently Requested

Hamartin expression and interaction with tuberin in tumor cell lines and primary cultures

Hamartin expression and interaction with tuberin in tumor cell lines and primary cultures

Journal of Neuroscience Research 63(3): 276-283

Tuberous sclerosis (TSC) is a neurocutaneous disorder characterized by multi-system hamartomatous lesions, and results from a mutation in TSC1, that encodes hamartin, or TSC2, that encodes tuberin. We have examined hamartin expression in a diverse range of human and rat cell lines and primary cultured cells derived from tissues that express hamartin in vivo. Strong hamartin signal was detected in every cell line of human origin examined, representing neuronal, epithelial, lymphoid, renal, vascular smooth muscle, liver, and prostatic cells. Primary cell cultures of oligodendroglioma, meningioma, and glioblastoma multiforme origin were also found to express hamartin. Hamartin was also detected in the rat PC12 cell line, as well as purified primary cultures of rat cortical neurons, astrocytes, and oligodendroglia, with a stronger signal found in astrocytes. Using co-immunoprecipitation, we have also confirmed the physical interaction of tuberin and hamartin in a diverse range of human and rat cell types. These findings demonstrate that hamartin is widely expressed in human and rat cell lines and cultures, and demonstrate that hamartin expression is not lost during the establishment of tumor cell lines or primary cultures. This suggests that the cell lines and cultures studied may serve as useful in vitro models for biochemical investigations involving hamartin and tuberin both individually and as a complex, as well as studies to elucidate the mechanisms underlying the organ-specific pathology of TSC.

(PDF emailed within 0-6 h: $19.90)

Accession: 046210474

Download citation: RISBibTeXText

PMID: 11170177

DOI: 10.1002/1097-4547(20010201)63:3<276::aid-jnr1021>3.0.co;2-a

Related references

Characterization of the cytosolic tuberin-hamartin complex. Tuberin is a cytosolic chaperone for hamartin. Journal of Biological Chemistry 274(50): 35647-35652, 1999

Expression of tuberin and hamartin in tuberous sclerosis complex-associated and sporadic cortical dysplasia of Taylor's balloon cell type. Folia Neuropathologica 46(1): 43-48, 2008

Pathological mutations of tuberin affect interaction with hamartin. American Journal of Human Genetics 67(4 Supplement 2): 406, October, 2000

Interaction between hamartin and tuberin, the TSC1 and TSC2 gene products. Human Molecular Genetics 7(6): 1053-1057, 1998

Distinct effects of single amino-acid changes to tuberin on the function of the tuberin-hamartin complex. European Journal of Human Genetics 13(1): 59-68, 2004

TSC2 missense mutations inhibit tuberin phosphorylation and prevent formation of the tuberin-hamartin complex. Human Molecular Genetics 10(25): 2889-2898, 2001

Tuberin and hamartin expression is reduced in the majority of subependymal giant cell astrocytomas in tuberous sclerosis complex consistent with a two-hit model of pathogenesis. Journal of Child Neurology 19(2): 102-106, 2004

Hamartin-Hsp70 interaction is necessary for Akt-dependent tuberin phosphorylation during heat shock. Bioscience, Biotechnology, and Biochemistry 73(11): 2488-2493, 2010

Pathological mutations in TSC1 and TSC2 disrupt the interaction between hamartin and tuberin. Human Molecular Genetics 10(25): 2899-2905, 2001

Hamartin and tuberin expression in human tissues. Modern Pathology 14(3): 202-210, 2001

Tuberin phosphorylation regulates its interaction with hamartin. Two proteins involved in tuberous sclerosis. Journal of Biological Chemistry 276(24): 21017-21021, 2001

Hamartin and tuberin interaction with the G2/M cyclin-dependent kinase CDK1 and its regulatory cyclins A and B. Journal of Neuropathology and Experimental Neurology 60(7): 711-723, 2001

Definition of domains mediating interaction between hamartin and tuberin and disruption by tuberous sclerosis associated mutations. Journal of Medical Genetics 37(Supplement 1): S23, September, 2000

Hamartin and tuberin immunohistochemical expression in cutaneous fibroepithelial polyps. Journal of Cutaneous Pathology 31(5): 383-387, 2004

Developmental expression of the tuberous sclerosis proteins tuberin and hamartin. Acta Neuropathologica 101(3): 202-210, March, 2001