- Caused by Proteases.
- Lysosomal Degradation of glycoproteins.
- Ubiquitim Proteasome Degradation of Proteins.
Before we start, please You must know about:
Present in Intestinal Track. 3 types of Proteases are:
- Trypsin:- Clearage at C-terminal of lys ad argimine e.g Asp à Lys à
- Chymotrypin:- Cleavage at C-Terminal of aromatic acids (Hydrophobic resides).
- Aminopeptidase:- Clearage at N-terminus.
- Carboxy Peptidases:- Cleavage at C-Terminus.
- Cleave large polypeptides to small peptidases.
Lysosomal degradation of Glycoproteins:
Proteins attach with carbohydrates Oligosaccharides Asn proteins.
ENZYME INVOLVED IN DEGRADATION OF OLIGOSACCHARIDES:
- Neuraminidase:- It attacks on acuity, the Neuraminic acid of Oligosaccharides chain deficiency of this enzyme results in sialidosis. They have mental disorder eye abnormalities, facial deformation.
- B-Galactosidase:- It attacks on B-Galactose. Deficiency of this enzyme leads to GM1 Gangliosidosis, MR (Mental Retarded) deformities other symptoms.
- Acetyl – Glucosaminidase:- It attacks on acetyl Glucosamine. Deficiency of this enzyme leads to tay sach/sand hoff. It causes mentally retarded bone deformities.
- α- Mannosidase:- It attacks on α Mannose. It’s deficiency leads to α-Mannosidosis. It causes mental retarded.
- β- Mannosidase:- It attacks on β-mannose. It’s deficiency leads to β-Mannosidosis. It causes mental retarded.
- Hexosaminidase:- It attacks on Acetate chitobiose (Present in Dog)… It is absent in human and rat. Instead of hexosaminidase humans and rat have chitobiose,.
- Chitobiase:- It attacks on Acetyl chitobiose. It’s deficiency causes no disorder present in human and rat.
- Fucosidase:- It attacks on fucose. It’s deficiency leads to fucosidosis. It causes mental retarded, heart muscles weaknesses.
The enzyme which attacks on Asn:
We have only 1 enzyme that attacks on Asn.
Glycosperginase:- It attacks Asn. It’s deficiency leads to Aspertylucosiminurla. Patients have metal retardation, severe diarrhea.
The enzyme which degrades Protein Part:
Cathepsins:- (A,B,C,D,H,L) 6 type of cathepsins degrade protein part. Active in the lysosome.
Ubiquitim Proteasome Degradation of Proteins:
Proteins which are degraded in the proteasome, firstly they are tagged with ubiquitin protein and they move toward proteasome for degradation.
Ubiquitin:- is a small protein of 8.5 xDa containing only 76 amino acids plays role in tagging or linking.