A branched oligosaccharide containing 

3 glucose (Glc)

9 mannose (Man)

2 N-acetylglucosamine (GlcNAc)

Glc3Man9(GlcNAc)2

TLS Online TPP Program

#Id: 8311

#Section 3: Genetics, Cellular and Molecular Biology

Actin,

basic building block of microfilaments extraordinarily well conserved among eukaryotes.

TLS Online TPP Program

#Id: 8226

#Section 3: Genetics, Cellular and Molecular Biology

I-cell disease

• almost all of the hydrolytic enzymes are missing from the lysosomes of many cell types.

• mis-sorting has been traced to a defective or missing GlcNAc phosphotransferase.

TLS Online TPP Program

#Id: 8225

#Section 3: Genetics, Cellular and Molecular Biology

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

TLS Online TPP Program

#Id: 8224

#Section 3: Genetics, Cellular and Molecular Biology

Lysosomal storage diseases:

• Hurler’s disease

• Inclusion-cell disease

TLS Online TPP Program

#Id: 8223

#Section 3: Genetics, Cellular and Molecular Biology

Retromer

a coat protein complex specialized for endosome-to-TGN transport, recycle the M6P receptor to the TGN

TLS Online TPP Program

#Id: 8221

#Section 3: Genetics, Cellular and Molecular Biology

The M6P receptor protein binds to M6P at pH 6.5–6.7 in the TGN lumen and releases it at pH 6, in the lumen of endosomes.