Page last updated: 2024-10-24

lysosomal transport

Definition

Target type: biologicalprocess

The directed movement of substances into, out of or within a lysosome. [GOC:ai]

Lysosomal transport is a complex process involving the delivery of macromolecules, organelles, and cellular debris to lysosomes for degradation. This process is essential for maintaining cellular homeostasis and preventing the accumulation of harmful substances.

**1. Sorting and Packaging:**
* Materials destined for lysosomes are first sorted and packaged into transport vesicles within the Golgi apparatus.
* This process involves the recognition of specific sorting signals on the target molecules by adaptor proteins.

**2. Vesicle Budding and Transport:**
* Once packaged, these transport vesicles bud off from the Golgi and travel towards the lysosomes.
* Motor proteins and microtubules within the cytoskeleton guide the vesicles along their path.

**3. Fusion with Early Endosomes:**
* Upon reaching the lysosomal pathway, transport vesicles fuse with early endosomes, which are small, membrane-bound organelles responsible for sorting and processing incoming material.
* The early endosome functions as a sorting hub, separating different cargo for delivery to various destinations.

**4. Maturation into Late Endosomes:**
* The early endosome then matures into a late endosome, gradually acidifying and acquiring hydrolytic enzymes.
* These enzymes, such as proteases, lipases, and nucleases, are responsible for breaking down the various components of the incoming material.

**5. Fusion with Lysosomes:**
* Finally, the late endosome fuses with a lysosome, a larger, more acidic organelle containing a complete set of hydrolytic enzymes.
* This fusion event creates a hybrid compartment where the degradation process takes place.

**6. Degradation and Recycling:**
* Inside the lysosome, the hydrolytic enzymes break down the incoming material into smaller molecules, such as amino acids, fatty acids, and sugars.
* These degradation products can be recycled back into the cytoplasm for use in other cellular processes.

**7. Autophagy:**
* Lysosomes are also involved in autophagy, a process that allows cells to degrade and recycle their own components.
* During autophagy, damaged organelles or portions of the cytoplasm are encapsulated in double-membrane vesicles called autophagosomes.
* These autophagosomes then fuse with lysosomes for degradation.

**8. Lysosomal Disorders:**
* Dysfunctional lysosomal transport can lead to a variety of inherited diseases, known as lysosomal storage disorders.
* These diseases arise from the accumulation of undigested material in lysosomes due to defects in specific hydrolytic enzymes or transport proteins.
* Examples include Pompe disease, Tay-Sachs disease, and Gaucher disease.

Lysosomal transport is a highly regulated process that plays a vital role in maintaining cellular homeostasis. Its intricate mechanisms ensure the efficient and timely delivery and degradation of cellular cargo, contributing to the overall health and function of the cell.'
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Proteins (3)

ProteinDefinitionTaxonomy
Proprotein convertase subtilisin/kexin type 9A proprotein convertase subtilisin/kexin type 9 that is encoded in the genome of human. [PRO:DNx, UniProtKB:Q8NBP7]Homo sapiens (human)
Heat shock 70 kDa protein 1A A heat shock 70 kDa protein 1A that is encoded in the genome of human. [PRO:DAN, UniProtKB:P0DMV8]Homo sapiens (human)
ProsaposinA prosaposin that is encoded in the genome of human. [PRO:DNx, UniProtKB:P07602]Homo sapiens (human)

Compounds (7)

CompoundDefinitionClassesRoles
adenosine diphosphateAdenosine Diphosphate: Adenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.adenosine 5'-phosphate;
purine ribonucleoside 5'-diphosphate
fundamental metabolite;
human metabolite
adenosinequinquefolan B: isolated from roots of Panax quinquefolium L.; RN not in Chemline 10/87; RN from Toxlitadenosines;
purines D-ribonucleoside
analgesic;
anti-arrhythmia drug;
fundamental metabolite;
human metabolite;
vasodilator agent
8-aminoadenosine
s 1033(trifluoromethyl)benzenes;
imidazoles;
pyridines;
pyrimidines;
secondary amino compound;
secondary carboxamide
anticoronaviral agent;
antineoplastic agent;
tyrosine kinase inhibitor
fenretinide4-hydroxyphenyl retinamide : A retinoid obtained by formal condensation of the carboxy group of all-trans retinoic acid and the anilino group of 4-hydroxyaniline. Synthetic retinoid agonist. Antiproliferative, antioxidant and anticancer agent with a long half-life in vivo. Apoptotic effects appear to be mediated by a mechanism distinct from that of 'classical' retinoids.

Fenretinide: A synthetic retinoid that is used orally as a chemopreventive against prostate cancer and in women at risk of developing contralateral breast cancer. It is also effective as an antineoplastic agent.
monocarboxylic acid amide;
retinoid
antineoplastic agent;
antioxidant
ver 155008VER 155008: structure in first sourcepurine nucleoside
PF-06446846PF-06446846 : A triazolopyridine that is 3H-[1,2,3]triazolo[4,5-b]pyridine substituted by a 4-{(3-chloropyridin-2-yl)[(3R)-piperidin-3-yl]carbamoyl}phenyl group at position 3. It is a potent inhibitor of PCSK9.

PF-06446846: inhibits translation of PCSK9 ;structure in first source
benzamides;
monochloropyridine;
piperidines;
tertiary carboxamide;
triazolopyridine
antilipemic drug;
EC 3.4.21.61 (kexin) inhibitor