Recent Insights into NCL Protein Function Using the Model Organism Dictyostelium discoideum
Abstract
:1. Neuronal Ceroid Lipofuscinosis
2. Studying the Functions of NCL Proteins Using the Model Organism Dictyostelium Discoideum
3. Using Dictyostelium to Study CLN2 Disease
3.1. Human TPP1
3.2. Loss of the Lysosomal Enzyme Tpp1A Impairs Autophagy and Multicellular Development in Dictyostelium
3.3. Tpp1B and Tpp1F Interact with the Golgi pH Regulator in Dictyostelium
4. Using Dictyostelium to Study CLN3 Disease
4.1. Human CLN3
4.2. Loss of Cln3 Causes Pleiotropic Effects in Dictyostelium that are Consistent with its Localization to the Contractile Vacuole System
4.3. Cln3 Regulates Osmoregulation in Dictyostelium
4.4. Cln3 Regulates Protein Secretion in Dictyostelium
5. Using Dictyostelium to Study CLN5 Disease
5.1. Human CLN5
5.2. Cln5 is Secreted and Functions as a Glycoside Hydrolase in Dictyostelium
5.3. Loss of Cln5 Impairs Adhesion and Chemotaxis during the Early Stages of Dictyostelium Development
6. Using Dictyostelium to Study the Molecular Networking of NCL Proteins
7. Conclusions
Author Contributions
Funding
Conflicts of Interest
Abbreviations
AprA | autocrine proliferation repressor A |
CadA | cell adhesion molecule A |
cAMP | 3′,5′-cyclic adenosine monophosphate |
CfaD | counting factor-associated protein D |
CLN | ceroid lipofuscinosis neuronal |
CMF | conditioned media factor |
CsaA | contact site A |
CtsD | cathepsin D |
CV | contractile vacuole |
ER | endoplasmic reticulum |
GPHR | Golgi pH regulator |
NCL | neuronal ceroid lipofuscinosis |
REMI | restriction enzyme-mediated integration |
RVD | regulatory volume decrease |
SCAR7 | spinocerebellar ataxia 7 |
SPPL | signal peptide peptidase-like |
StpA | suppressor of Tpp1 A |
TPP1 | tripeptidyl peptidase 1 |
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dictyBase ID | Protein Names | Gene Names |
---|---|---|
DDB0231036 | Autocrine proliferation repressor protein A (PhoPQ-activated pathogenicity-related protein) | aprA DDB_G0281663 |
DDB0215012 | Cathepsin D (Ddp44) | ctsD, catD DDB_G0279411 |
DDB0214999 | Cysteine proteinase 4 | cprD, CP4 DDB_G0278721 |
DDB0185092 | Cysteine proteinase 5 | cprE, CP5 DDB_G0272815 |
DDB0215005 | Cysteine proteinase 7 | cprG, CP7 DDB_G0279187 |
DDB0191134 | Elongation factor 1-alpha (EF-1-alpha) (50 kDa actin-binding protein) (ABP-50) | eef1a2, efaa2, efaAII DDB_G0269136 |
DDB0233663 | Luminal-binding protein (BiP 2) | bip2 DDB_G0276445 |
DDB0349243 | Uncharacterized protein | DDB_G0288563 |
DDB0233868 | Uncharacterized protein, member of the peptidase S28 family of serine proteases, a group containing lysosomal Pro-X carboxypeptidase, dipeptidyl-peptidase II, and thymus-specific serine peptidase | DDB_G0289749 |
DDB0238155 | Induced after Legionella infection Contains a putative N-terminal signal sequence; regulated by gskA and zakA; induced by Legionella pneumophila infection | iliA DDB_G0285615 |
Phenotype | Tpp1a− | Cln3− | Cln5− |
---|---|---|---|
Increased cell proliferation | No | Yes | Not known |
Impaired cytokinesis | Not known | Yes | Not known |
Autofluorescent inclusions | Yes | Not known | Yes |
Defects in osmoregulation | Not known | Yes | Not known |
Aberrant protein secretion | Not known | Yes | Not known |
Reduced adhesion | Not known | Yes | Yes |
Function linked to autophagy | Yes | Not known | Yes |
Precocious development | Yes | Yes | Not known |
Impaired spore formation | Yes | Not known | Not known |
Reduced spore viability/integrity | No | Yes | Not known |
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McLaren, M.D.; Mathavarajah, S.; Huber, R.J. Recent Insights into NCL Protein Function Using the Model Organism Dictyostelium discoideum. Cells 2019, 8, 115. https://doi.org/10.3390/cells8020115
McLaren MD, Mathavarajah S, Huber RJ. Recent Insights into NCL Protein Function Using the Model Organism Dictyostelium discoideum. Cells. 2019; 8(2):115. https://doi.org/10.3390/cells8020115
Chicago/Turabian StyleMcLaren, Meagan D., Sabateeshan Mathavarajah, and Robert J. Huber. 2019. "Recent Insights into NCL Protein Function Using the Model Organism Dictyostelium discoideum" Cells 8, no. 2: 115. https://doi.org/10.3390/cells8020115