Hurler’s disease- mutation in a structural gene that codes for an individual lysosomal hydrolase.

TLS Online TPP Program

#Id: 8191

#XL - Q Biochemistry

PTS1 signals are recognized by the peroxin Pex5 receptor

PTS2 signals are recognized by the peroxin Pex7 receptor

Neither targeting sequence is cleaved after import.

TLS Online TPP Program

#Id: 8190

#XL - Q Biochemistry

Peroxins

Catalyse peroxisomal protein import by ATP hydrolysis.

TLS Online TPP Program

#Id: 8189

#XL - Q Biochemistry

C-terminal peroxisomal signal sequences Ser–Lys–Leu (PTS1)

N-terminal peroxisomal signal sequences slightly longer and partially hydrophobic (PTS2)

TLS Online TPP Program

#Id: 8188

#XL - Q Biochemistry

Plasmalogens

• most abundant class of PL in myelin sheaths

• Analogous to PC

• Contain an unusual fatty alcohol that is attached through an ether linkage.

TLS Online TPP Program

#Id: 8187

#XL - Q Biochemistry

Biosynthetic function of animal peroxisomes- 

Synthesis of cholesterol and dolichol 

Synthesis of bile acids in the liver 

Catalyze the first reactions in the formation of Plasmalogens

TLS Online TPP Program

#Id: 8186

#XL - Q Biochemistry

Glyoxysomes

Conversion of fats to sugars is accomplished by a series of reactions known as the Glyoxylate cycle