Hurler’s disease- mutation in a structural gene that codes for an individual lysosomal hydrolase.

TLS Online TPP Program

#Id: 9365

#XL - T Zoology

Inside the nucleus, R smad-co smad complex binds to nuclear transcription factor TFE3 to activate transcription of PAI-1 & other ECM proteins such as fibronectin & collagens

TLS Online TPP Program

#Id: 9366

#XL - T Zoology

Within the nucleus, R-Smads are modified by:

Phosphorylation of linker domains

Acetylation of MH1 domains

Dephosphorylation of the C-terminal serines by nuclear

phosphatases

TLS Online TPP Program

#Id: 9367

#XL - T Zoology

BMP proteins, activate a different set of receptors that are similar to the TGF-β RI and RII proteins but phosphorylate other R-Smads, Smad1, Smad5, & Smad8

TLS Online TPP Program

#Id: 9368

#XL - T Zoology

Smad4 was originally called DPC (deleted in pancreatic cancer)

TLS Online TPP Program

#Id: 9369

#XL - T Zoology

Negative regulators of TGF-β signaling are induced by TGF-β signaling (Example of Negative feedback): 

Ski 

SnoN 

I-Smads (Smad7)

TLS Online TPP Program

#Id: 9370

#XL - T Zoology

SnoN and Ski trigger abnormal cell proliferation by blocking the Smad-complex.