Tuberous Sclerosis Complex 1 Protein

An intracellular signaling and tumor suppressor protein that forms a complex with TUBEROUS SCLEROSIS COMPLEX 2 PROTEIN (TSC2) and other signaling factors to negatively regulate MTORC1 signaling and affect cell growth and proliferation. Structurally, it interacts with TSC2 through its N-terminal, which also contains GSK-3BETA phosphorylation sites and a RHO-KINASE activation domain. It also contains a C-terminal coiled-coil domain and ezrin-radixin-moesin (ERM) domain. Mutations in the TSC1 gene are associated with TUBEROUS SCLEROSIS.

Also Known As:

Hamartin

Networked: 158 relevant articles (5 outcomes, 9 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Amino Acids, Peptides, and Proteins: 1
- Proteins: 484843
  - Intracellular Signaling Peptides and Proteins: 32
    - Tuberous Sclerosis Complex 1 Protein: 158
  - Neoplasm Proteins: 2
    - Tumor Suppressor Proteins: 1683
      - Tuberous Sclerosis Complex 1 Protein: 158
        human TSC1 protein
        mouse Tsc1 protein
        rat Tsc1 protein
- Peptides: 82426
  - Intracellular Signaling Peptides and Proteins: 32
    - Tuberous Sclerosis Complex 1 Protein: 158

Experts

1.	Blenis, John: 6 articles (02/2022 - 10/2002)
2.	Kotulska, Katarzyna: 6 articles (01/2018 - 02/2004)
3.	Tee, Andrew R: 5 articles (02/2022 - 10/2002)
4.	Kwiatkowski, David J: 5 articles (01/2021 - 10/2002)
5.	Hengstschläger, Markus: 5 articles (07/2009 - 12/2003)
6.	Rosner, Margit: 5 articles (07/2009 - 12/2003)
7.	Cantley, Lewis C: 4 articles (02/2022 - 10/2002)
8.	Manning, Brendan D: 4 articles (02/2022 - 10/2002)
9.	Prabhakar, Shilpa: 4 articles (01/2021 - 12/2005)
10.	Ramesh, Vijaya: 4 articles (01/2021 - 11/2002)

Related Diseases

1.	Tuberous Sclerosis (Bourneville's Disease) 12/13/2019 - "Long-Term Therapeutic Efficacy of Intravenous AAV-Mediated Hamartin Replacement in Mouse Model of Tuberous Sclerosis Type 1." 10/01/2020 - "Our study demonstrated that the novel TSC1 frameshift mutation (TSC1 c.1550_1551del) trigger aberrant splicing and NMD simultaneously, causing decrease of hamartin, then, leading to tuberous sclerosis complex formation." 01/01/2020 - "Tuberous sclerosis complex 1 (TSC1), also known as hamartin, is thought to be one such molecule. " 10/01/2015 - "Survival benefit and phenotypic improvement by hamartin gene therapy in a tuberous sclerosis mouse brain model." 03/01/2009 - "Dissociate expression of tuberous sclerosis complex 1 product hamartin in a skin and pulmonary lesion of a tuberous sclerosis complex."
2.	Epilepsy (Aura) 03/01/2016 - "Among the combinatorial targets were TSC1, coding for the protein hamartin, and several epilepsy risk genes. " 08/01/2013 - "Understanding the endogenous mechanism for neuroprotection mediated by hamartin may afford a novel approach to effective treatment of neurological diseases such as stroke, neurodegenerative diseases, and epilepsy, with possible applications to nonneurological conditions. " 09/01/2011 - "Recently, understanding the neurobiology of hamartin and tuberin in the development of epilepsy and cognitive impairment associated with tuberous sclerosis complex allowed the development of sirolimus and everolimus to be used in human clinical trials. "
3.	Neoplasm Metastasis (Metastasis) 07/01/2005 - "Likewise, hamartin levels were significantly lower in patients with metastasis, recurrence and mortality, when compared with those remained disease free (P = 0.001, 0.041 and 0.003, respectively). " 12/01/2003 - "Hamartin and tuberin may play functional roles in the suppression of cell migration and/or metastasis, possibly through their regulation of the small GTPase Rho."
4.	Stroke (Strokes) 08/01/2013 - "Understanding the endogenous mechanism for neuroprotection mediated by hamartin may afford a novel approach to effective treatment of neurological diseases such as stroke, neurodegenerative diseases, and epilepsy, with possible applications to nonneurological conditions. "
5.	Neurodegenerative Diseases (Neurodegenerative Disease) 08/01/2013 - "Understanding the endogenous mechanism for neuroprotection mediated by hamartin may afford a novel approach to effective treatment of neurological diseases such as stroke, neurodegenerative diseases, and epilepsy, with possible applications to nonneurological conditions. "

Related Drugs and Biologics

1.	Tuberous Sclerosis Complex 2 Protein
2.	Proteins (Proteins, Gene)
3.	Sirolimus (Rapamycin)
4.	AMP-Activated Protein Kinases
5.	Vascular Endothelial Growth Factor A (Vascular Endothelial Growth Factor)
6.	70-kDa Ribosomal Protein S6 Kinases (Ribosomal Protein S6 Kinases, 70 kDa)
7.	Insulin (Novolin)
8.	Biomarkers (Surrogate Marker)
9.	Protein Kinases (Protein Kinase)
10.	Everolimus

Related Therapies and Procedures

1.	Kidney Transplantation
2.	Therapeutics