Term
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Definition
DUB inhibitor.
Inhibits ubiquitin-cleaving isopeptidases. |
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Term
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Definition
| Protein involved in translocation: cytosol to ER. |
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Term
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Definition
Hexameric AAA-ATPase. Thought to be involved in the dislocation of ERAD substrates from the ER membrane.
Inhibited by NMS-873 and Eeyarestatin. |
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Term
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Definition
Performs the first step in triglyceride hydrolysis. TAG -> DAG + FFAs.
Stimulated by other lipases (e.g. pancreatic lipase, LPL) |
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Term
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Definition
Co-activator of ATGL. Loss of CGI-58 causes systemic TG accumulation (meaning it's important for stimulating lipolysis).
Is present on lipid droplets. |
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Term
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Definition
| An inhibitor of ATGL. Is present on lipid droplets. |
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Term
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Definition
Promote ubiquitylation without forming a covalent intermediate with ubiquitin.
CUL1: substrate receptors recruited by SKP1. |
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Term
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Definition
A multi-subunit ubiquitin ligase.
Consists of SKP1, CUL1, and an F-box protein that confers substrate specificity as well as a RING protein known as RBX1. |
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Term
| HECT-Domain ubiquitin ligases |
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Definition
A class of E3 ubiquitin ligases.
Forms a covalent intermediate with ubiquitin (strictly conserved cysteine residue that forms a thiol ester intermediate). |
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Term
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Definition
A ubiquitin-like protein.
Neddylation of CUL1 enhances CUL1-dependent ub-ligase activity, possibly by recruiting ub-loaded E2s.
Deneddylation of cullins (CUL1, 2, 3) decreases recruitment/activation of E2s. |
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Term
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Definition
Small ubiquitin-related modifier (family of proteins).
Promotes ubiquitination of ERAD substrates by an E3 (RNF4).
Covalently attaches to other proteins.
Control pathway is similar to ubiquitin pathway.
Can act by blocking ubiquitin attachment sites (attaches to isopeptide lysines). |
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Term
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Definition
| In yeast, ERAD L/M. Doa10p: ERAD-C. |
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Term
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Definition
| Glucose regulated protein 78. Involved in proper protein folding in the ER. |
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Term
| Calreticulin and Calnexin |
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Definition
| Chaperones that assist in folding of glycoproteins in the ER (post-translational modification). |
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Term
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Definition
| ER-luminal lectins. They bind selectively to N-glycans bearing an a-1,6-linked mannose and to SEL1L, a subunit of Hrd1. |
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Term
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Definition
A subunit of Hrd1 complex.
Required for release of substrates from OS-9 to downstream ERAD components (e.g. Hrd1). |
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Term
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Definition
ERAD factors; implicated as structural components of the dislocon that interact directly with translocating substrates.
Derlin-1: brings together the pulling ATPase (VCP) and ubiquitination ligases (Hrd1, gp78 |
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Term
| SREBPs and cholesterol synthesis: mechanism |
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Definition
Mmb-bound transcription factors.
After synthesis in ER, SREBPs form a complex with SCAP (inactive). When cholesterol levels are low, SCAP escorts SREBP (ER->Golgi) for cleavage, thus activating nuclear transcription of HMGR.
When cholesterol is high, SCAP-SREBP is retained in the ER. |
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Term
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Definition
ER-membrane proteins; they regulate SREBP proteolytic processing.
Cholesterol is high: SCAP binds to Insigs, blocking SCAP-SREBP movement from ER->Golgi and decreasing lipid synthesis.
Insig-1 is ubiquitinated by gp78 (E3 ub-ligase). |
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Term
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Definition
ERAD protein, localizes to ER and LDs; its depletion impairs substrate degradation.
Recruits UBE2G2 (E2 enzyme).
AUP1's CUE domain regulates polyubiquitylation and facilitates AUP1's interaction with HRD1 complex. |
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Term
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Definition
-Sterols accumulate in ER membrane
-Triggers binding of HMG-CoA reductase to ER mmb proteins Insig-1 and -2
-Insig binding leads to recrtuiment of gp78 (E3 ligase), initiating ubiquitination of HMG-CoA reductase |
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Term
| US2 and US11 genes (HCMV gene products) |
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Definition
| They encode ER-resident membrane glycoproteins. They bind newly synthesized MHC-I in the ER lumen. They initiate dislocation of MHC-I from the ER back to the cytosol before proteasome-mediated degradation. |
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Term
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Definition
| A DUB; component of the p97 complex via its UBX domain. Interacts with Der1 and UBXD8. |
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