JavaScript is disabled in your browser. Please enable JavaScript to view this website.
AB152772

重组人Hamartin蛋白

Recombinant Human Hamartin protein

Be the first to review this product! Submit a review

|

(0 Publication)

Recombinant Human Hamartin protein is a Human Fragment protein, in the 166 to 274 aa range, expressed in Wheat germ, suitable for SDS-PAGE, ELISA, WB.

查看别名

KIAA0243, TSC, TSC1, Hamartin, Tuberous sclerosis 1 protein

1 Images
SDS-PAGE - Recombinant Human Hamartin protein (AB152772)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human Hamartin protein (AB152772)

12.5% SDS-PAGE analysis of ab152772 stained with Coomassie Blue.

关键信息

表达系统

Wheat germ

标签

Tag free

应用

ELISA, WB, SDS-PAGE

applications

生物活性

No

访问

Q92574

不含动物源

No

不含载体蛋白

No

种属

Human

存储溶液

pH: 8 Constituents: 0.79% Tris HCl, 0.31% Glutathione

storage-buffer

style
left-column

反应性数据

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "ELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "WB": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
style
left-column
style
left-column

序列信息

[{"sequence":"LKKPGHVAEVYLVHLHASVYALFHRLYGMYPCNFVSFLRSHYSMKENLETFEEVVKPMMEHVRIHPELVTGSKDHELDPRRWKRLETHDVVIECAKISLDPTEASYEDG","proteinLength":"Fragment","predictedMolecularWeight":"37.73 kDa","actualMolecularWeight":null,"aminoAcidEnd":274,"aminoAcidStart":166,"nature":"Recombinant","expressionSystem":"Wheat germ","accessionNumber":"Q92574","tags":[]}]
style
left-column

性能和储存信息

运输条件
Dry Ice
推荐的短期储存条件
-80°C
推荐的长期储存条件
-80°C
分装信息
Upon delivery aliquot
储存信息
Avoid freeze / thaw cycle
False
style
left-column

补充信息

This supplementary information is collated from multiple sources and compiled automatically.

The protein 'Hamartin' also known as TSC1 plays a mechanical role in cellular processes primarily as a regulator of cellular growth and proliferation. It interacts with Tuberin (TSC2) to form a complex that inhibits the small GTPase Rheb thereby controlling the mTOR signaling pathway. Hamartin has a molecular mass of approximately 130 kDa. It is expressed in various tissues including the brain heart and kidneys highlighting its significance in multiple physiological processes.
Biological function summary

Hamartin functions as part of the TSC1-TSC2 complex which acts as a tumor suppressor. This complex inhibits the mechanistic target of rapamycin complex 1 (mTORC1) an essential regulator of cell growth proliferation and protein synthesis. Through this regulation Hamartin plays a critical role in maintaining cellular homeostasis and preventing abnormal cell growth that can lead to tumorigenesis.

Pathways

Hamartin is integral to the mTOR signaling pathway an important pathway that regulates cell metabolism growth and survival. This pathway involves the interaction of Hamartin with Tuberin (TSC2) helping to control the activation of mTORC1. Additionally Hamartin is involved in the PI3K/Akt signaling pathway which further influences mTOR activity and cellular responses to growth factors.

Disruptions in Hamartin function are linked to Tuberous Sclerosis Complex (TSC) a genetic disorder characterized by tumor development in multiple organs. Mutations in the TSC1 gene lead to the loss of Hamartin function resulting in uncontrolled cell growth. Additionally research indicates that Hamartin may have a connection to certain epilepsy types given its expression in the brain and its role in neuronal development. In these conditions the interaction of Hamartin with Tuberin is essential for regulating cellular behavior and preventing disease progression.
style
left-column

特殊说明

形式

Liquid

style
left-column

常规信息

功能

Non-catalytic component of the TSC-TBC complex, a multiprotein complex that acts as a negative regulator of the canonical mTORC1 complex, an evolutionarily conserved central nutrient sensor that stimulates anabolic reactions and macromolecule biosynthesis to promote cellular biomass generation and growth (PubMed : 12172553, PubMed : 12271141, PubMed : 12906785, PubMed : 15340059, PubMed : 24529379, PubMed : 28215400). The TSC-TBC complex acts as a GTPase-activating protein (GAP) for the small GTPase RHEB, a direct activator of the protein kinase activity of mTORC1 (PubMed : 12906785, PubMed : 15340059, PubMed : 24529379). In absence of nutrients, the TSC-TBC complex inhibits mTORC1, thereby preventing phosphorylation of ribosomal protein S6 kinase (RPS6KB1 and RPS6KB2) and EIF4EBP1 (4E-BP1) by the mTORC1 signaling (PubMed : 12271141, PubMed : 24529379, PubMed : 28215400, PubMed : 33215753). The TSC-TBC complex is inactivated in response to nutrients, relieving inhibition of mTORC1 (PubMed : 12172553, PubMed : 24529379). Within the TSC-TBC complex, TSC1 stabilizes TSC2 and prevents TSC2 self-aggregation (PubMed : 10585443, PubMed : 28215400). Acts as a tumor suppressor (PubMed : 9242607). Involved in microtubule-mediated protein transport via its ability to regulate mTORC1 signaling (By similarity). Also acts as a co-chaperone for HSP90AA1 facilitating HSP90AA1 chaperoning of protein clients such as kinases, TSC2 and glucocorticoid receptor NR3C1 (PubMed : 29127155). Increases ATP binding to HSP90AA1 and inhibits HSP90AA1 ATPase activity (PubMed : 29127155). Competes with the activating co-chaperone AHSA1 for binding to HSP90AA1, thereby providing a reciprocal regulatory mechanism for chaperoning of client proteins (PubMed : 29127155). Recruits TSC2 to HSP90AA1 and stabilizes TSC2 by preventing the interaction between TSC2 and ubiquitin ligase HERC1 (PubMed : 16464865, PubMed : 29127155).

翻译后修饰

Phosphorylation at Ser-505 does not affect interaction with TSC2.. 'Lys-63'-linked ubiquitinated at Lys-30 by PELI1; the ubiquitination promotes TSC1/TSC2 complex stability.

style
left-column

产品实验方案

style
left-column

靶点信息

Non-catalytic component of the TSC-TBC complex, a multiprotein complex that acts as a negative regulator of the canonical mTORC1 complex, an evolutionarily conserved central nutrient sensor that stimulates anabolic reactions and macromolecule biosynthesis to promote cellular biomass generation and growth (PubMed : 12172553, PubMed : 12271141, PubMed : 12906785, PubMed : 15340059, PubMed : 24529379, PubMed : 28215400). The TSC-TBC complex acts as a GTPase-activating protein (GAP) for the small GTPase RHEB, a direct activator of the protein kinase activity of mTORC1 (PubMed : 12906785, PubMed : 15340059, PubMed : 24529379). In absence of nutrients, the TSC-TBC complex inhibits mTORC1, thereby preventing phosphorylation of ribosomal protein S6 kinase (RPS6KB1 and RPS6KB2) and EIF4EBP1 (4E-BP1) by the mTORC1 signaling (PubMed : 12271141, PubMed : 24529379, PubMed : 28215400, PubMed : 33215753). The TSC-TBC complex is inactivated in response to nutrients, relieving inhibition of mTORC1 (PubMed : 12172553, PubMed : 24529379). Within the TSC-TBC complex, TSC1 stabilizes TSC2 and prevents TSC2 self-aggregation (PubMed : 10585443, PubMed : 28215400). Acts as a tumor suppressor (PubMed : 9242607). Involved in microtubule-mediated protein transport via its ability to regulate mTORC1 signaling (By similarity). Also acts as a co-chaperone for HSP90AA1 facilitating HSP90AA1 chaperoning of protein clients such as kinases, TSC2 and glucocorticoid receptor NR3C1 (PubMed : 29127155). Increases ATP binding to HSP90AA1 and inhibits HSP90AA1 ATPase activity (PubMed : 29127155). Competes with the activating co-chaperone AHSA1 for binding to HSP90AA1, thereby providing a reciprocal regulatory mechanism for chaperoning of client proteins (PubMed : 29127155). Recruits TSC2 to HSP90AA1 and stabilizes TSC2 by preventing the interaction between TSC2 and ubiquitin ligase HERC1 (PubMed : 16464865, PubMed : 29127155).
See full target information TSC1
style
left-column
style
left-column
style
right-column

搭配使用产品

Proteins & Peptides

AB56271

Recombinant Human RHEB protein

SDS-PAGE - Recombinant Human RHEB protein (AB56271)

  • 0
  • 0
Primary Antibodies

AB270967

Anti-Hamartin antibody [EPR24364-109]

20ul selling size|KO Validated|RabMAb|Recombinant|Trial Size

Immunocytochemistry/ Immunofluorescence - Anti-Hamartin antibody [EPR24364-109] (AB270967)

  • 0
  • 0
Proteins & Peptides

AB114179

Recombinant Human mTOR protein

SDS-PAGE - Recombinant Human mTOR protein (AB114179)

  • 0
  • 0
Proteins & Peptides

AB167933

Recombinant Human S6K1 protein

SDS-PAGE - Recombinant Human S6K1 protein (AB167933)

  • 0
  • 0

Abcam Product Promise

我们致力于为您的研究提供高质量的试剂，为您科研的每一步提供支持。若我们的产品未能达到预期性能，我们向您提供 Abcam Product Promise 保障。
详情请参阅我们的条款与条件。

Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.

For licensing inquiries, please contact partnerships@abcam.com