GM2 activator

GM2 activator
	Belt model according to PDB 1G13
Properties of human protein
Mass / length primary structure	162 amino acids
Precursor	(170 aa)
Isoforms	Short (160 aa)
Identifier
Gene name	GM2A
External IDs	OMIM : 613109 ; UniProt : P17900 ;
Occurrence
Parent taxon	Vertebrates

The GM2 activator is a protein that occurs in vertebrates and is necessary for the breakdown of gangliosides , special lipids in the body. This breakdown takes place in lysosomes within the cells , where the GM2 activator can be found. Gm2a allows the extraction of individual GM2 molecules from the membrane , binds GM2 and presents it to the GM2-degrading enzyme hexosaminidase A . In humans, mutations in GM2a - gene , the cause of GM2 Gangliosidosis type AB .

GM2 ganglioside

GM2 and other lipids are not soluble in water. In order to be able to process them with the (water-soluble) enzymes, they or their hydrophobic parts must be embedded in a hydrophobic bubble that is part of the GM2 activator. The GM2 activator recognizes the neuraminic acid residues in gangliosides, so that specifically only these lipids are absorbed from the membrane.

Due to its properties, the GM2 activator could generally function as a transport protein for glycosphingolipids. An increased expression of the GM2 activator, caused by oxidized LDL, could be the cause of local accumulation of glycosphingolipids in arteriosclerosis.

literature

Y. Ran, GE Fanucci: Ligand extraction properties of the GM2 activator protein and its interactions with lipid vesicles. In: Biophysical Journal. Volume 97, Number 1, July 2009, pp. 257-266. doi : 10.1016 / j.bpj.2009.03.065 . PMID 19580763 . PMC 2711380 (free full text).
M. Wendeler, N. Werth et al. a .: The enzyme-binding region of human GM2-activator protein. In: The FEBS Journal . Volume 273, Number 5, March 2006, pp. 982-991. doi : 10.1111 / j.1742-4658.2006.05126.x . PMID 16478472 .
CS Wright, Q. Zhao, F. Rastinejad: Structural analysis of lipid complexes of GM2-activator protein. In: Journal of molecular biology . Volume 331, Number 4, August 2003, pp. 951-964. PMID 12909021 .

Individual evidence

↑ Orthologist at OrthoDB
↑ UniProt P17900
^ Y. Hama, YT Li, SC Li: Interaction of GM2 activator protein with glycosphingolipids. In: The Journal of biological chemistry . Volume 272, Number 5, January 1997, pp. 2828-2833. PMID 9006924 .
↑ DJ Mahuran: The GM2 activator protein, its roles as a co-factor in GM2 hydrolysis and as a general glycolipid transport protein. In: Biochimica et biophysica acta . Volume 1393, Number 1, July 1998, pp. 1-18. PMID 9714704 . (Review).
↑ H. Yanai, H. Yoshida et al. a .: The possible contribution of a general glycosphingolipid transporter, GM2 activator protein, to atherosclerosis. In: Journal of atherosclerosis and thrombosis. Volume 13, Number 6, December 2006, pp. 281-285, ISSN 1340-3478 . PMID 17192692 . (Review).

[1] Orthologist at OrthoDB

[2] UniProt P17900

[PMID9006924-3] Y. Hama, YT Li, SC Li: Interaction of GM2 activator protein with glycosphingolipids. In: The Journal of biological chemistry . Volume 272, Number 5, January 1997, pp. 2828-2833. PMID 9006924 .

[PMID9714704-4] DJ Mahuran: The GM2 activator protein, its roles as a co-factor in GM2 hydrolysis and as a general glycolipid transport protein. In: Biochimica et biophysica acta . Volume 1393, Number 1, July 1998, pp. 1-18. PMID 9714704 . (Review).

[PMID17192692-5] H. Yanai, H. Yoshida et al. a .: The possible contribution of a general glycosphingolipid transporter, GM2 activator protein, to atherosclerosis. In: Journal of atherosclerosis and thrombosis. Volume 13, Number 6, December 2006, pp. 281-285, ISSN 1340-3478 . PMID 17192692 . (Review).