lysosomal enzymes - определение. Что такое lysosomal enzymes
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Что (кто) такое lysosomal enzymes - определение

SMALL LYTIC VACUOLE WITH CELL CYCLE-INDEPENDENT MORPHOLOGY, FOUND IN MOST ANIMAL CELLS; CONTAINS A VARIETY OF HYDROLASES, MOST OF WHICH HAVE THEIR MAXIMAL ACTIVITIES IN THE PH RANGE 5-6
Lyosome; Lysosomes; Lysozome; Lysosomal; Lysosomal enzymes; Lyosomes; Enzymes in lysosomes; Lysosome(s); Lysome
  • The lysosome is shown in purple, as an endpoint in endocytotic sorting. AP2 is necessary for vesicle formation, whereas the mannose-6-receptor is necessary for sorting hydrolase into the lysosome's lumen.
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  • TEM views of various vesicular compartments. Lysosomes are denoted by "Ly". They are dyed dark due to their acidity; in the center of the top image, a Golgi Apparatus can be seen, distal from the cell membrane relative to the lysosome .

lysosome         
['l??s?s??m]
¦ noun Biology an organelle in the cytoplasm of eukaryotic cells containing degradative enzymes enclosed in a membrane.
Derivatives
lysosomal adjective
Lysosome         
A lysosome () is a membrane-bound organelle found in many animal cells.By convention similar cells in plants are called vacuoles, see They are spherical vesicles that contain hydrolytic enzymes that can break down many kinds of biomolecules.
Lysosomal storage disease         
INHERITED METABOLIC DISORDER THAT INVOLVE AN ABNORMAL ACCUMULATION OF SUBSTANCES INSIDE THE LYSOSOME RESULTING FROM DEFECTS IN LYSOSOMAL FUNCTION
Lysosomal storage disorder; Lysosomal storage diseases; Lysosomal storage diseases, nervous system; Lysosomal disorders; Lysosomal storage disorders; Lysosomal Storage Disease; Lysosomal disorder; Lysosomal disease
Lysosomal storage diseases (LSDs; ) are a group of over 70 rare inherited metabolic disorders that result from defects in lysosomal function. Lysosomes are sacs of enzymes within cells that digest large molecules and pass the fragments on to other parts of the cell for recycling.

Википедия

Lysosome

A lysosome () is a membrane-bound organelle found in many animal cells. They are spherical vesicles that contain hydrolytic enzymes that can break down many kinds of biomolecules. A lysosome has a specific composition, of both its membrane proteins, and its lumenal proteins. The lumen's pH (~4.5–5.0) is optimal for the enzymes involved in hydrolysis, analogous to the activity of the stomach. Besides degradation of polymers, the lysosome is involved in various cell processes, including secretion, plasma membrane repair, apoptosis, cell signaling, and energy metabolism.

Lysosomes are termed to be degradative organelles that act as the waste disposal system of the cell by digesting used materials in the cytoplasm, from both inside and outside the cell. Material from outside the cell is taken up through endocytosis, while material from the inside of the cell is digested through autophagy. The sizes of the organelles vary greatly—the larger ones can be more than 10 times the size of the smaller ones. They were discovered and named by Belgian biologist Christian de Duve, who eventually received the Nobel Prize in Physiology or Medicine in 1974.

Lysosomes are known to contain more than 60 different enzymes, and have more than 50 membrane proteins. Enzymes of the lysosomes are synthesized in the rough endoplasmic reticulum and exported to the Golgi apparatus upon recruitment by a complex composed of CLN6 and CLN8 proteins. The enzymes are trafficked from the Golgi apparatus to lysosomes in small vesicles, which fuse with larger acidic vesicles. Enzymes destined for a lysosome are specifically tagged with the molecule mannose 6-phosphate, so that they are properly sorted into acidified vesicles.

In 2009, Marco Sardiello and co-workers discovered that the synthesis of most lysosomal enzymes and membrane proteins is controlled by transcription factor EB (TFEB), which promotes the transcription of nuclear genes. Mutations in the genes for these enzymes are responsible for more than 50 different human genetic disorders, which are collectively known as lysosomal storage diseases. These diseases result from an accumulation of specific substrates, due to the inability to break them down. These genetic defects are related to several neurodegenerative disorders, cancers, cardiovascular diseases, and aging-related diseases.