PEX7 binds cargo proteins containing PTS2

Stable Identifier
Reaction [binding]
Homo sapiens
Locations in the PathwayBrowser
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

Cytosolic PEX7 binds peroxisome targeting signal 2 (PTS2), a sequence of nine amino acid residues functionally identified in 3 human peroxisomal matrix proteins (Braverman et al. 1997, Motley et al. 1997, Purdue et al. 1997, Braverman et al. 2000, Ghys et al. 2002, Motley et al. 2002, Kunze et al. 2011, Kunze et al. 2015). According to molecular modeling, the PTS2 consensus sequence binds a groove in PEX7 (Kunze et al. 2011). Mutations in PEX7 cause rhizomelic chondrodysplasia punctata type 1 (Braverman et al. 1997, Motley et al. 1997, Purdue et al. 1997).

Literature References
PubMed ID Title Journal Year
9090383 Rhizomelic chondrodysplasia punctata is caused by deficiency of human PEX7, a homologue of the yeast PTS2 receptor

Lazarow, PB, Zhang, JW, Skoneczny, M, Purdue, PE

Nat. Genet. 1997
11781871 Mutational spectrum in the PEX7 gene and functional analysis of mutant alleles in 78 patients with rhizomelic chondrodysplasia punctata type 1

Haasjes, J, Tabak, HF, Gerez, L, Benne, R, Brites, P, Motley, AM, Hogenhout, E, Waterham, HR, Wanders, RJA

Am. J. Hum. Genet. 2002
9090381 Human PEX7 encodes the peroxisomal PTS2 receptor and is responsible for rhizomelic chondrodysplasia punctata

Gould, SJ, Steel, G, Braverman, N, Moser, A, Obie, C, Moser, H, Valle, D

Nat. Genet. 1997
25538232 Mechanistic insights into PTS2-mediated peroxisomal protein import: the co-receptor PEX5L drastically increases the interaction strength between the cargo protein and the receptor PEX7

Schmid, JA, Maurer-Stroh, S, Malkani, N, Berger, J, Wiesinger, C, Kunze, M

J. Biol. Chem. 2015
10673331 PEX7 gene structure, alternative transcripts, and evidence for a founder haplotype for the frequent RCDP allele, L292ter

Steel, G, Braverman, N, Moser, A, Moser, H, Valle, D, Lin, P

Genomics 2000
11931631 Functional studies on human Pex7p: subcellular localization and interaction with proteins containing a peroxisome-targeting signal type 2 and other peroxins

Van Veldhoven, PP, Ghys, K, Mannaerts, GP, Fransen, M

Biochem. J. 2002
9090382 Rhizomelic chondrodysplasia punctata is a peroxisomal protein targeting disease caused by a non-functional PTS2 receptor

Brites, P, Wijburg, FA, Distel, B, Heijmans, HS, Motley, AM, Tabak, HF, ten Asbroek, AL, Hettema, EH, Baas, F, Wanders, RJ, Hogenhout, EM

Nat. Genet. 1997
22057399 Structural requirements for interaction of peroxisomal targeting signal 2 and its receptor PEX7

Neuberger, G, Schmid, JA, Eck, T, Maurer-Stroh, S, Berger, J, Kunze, M, Ma, J, Eisenhaber, F, Braverman, N

J. Biol. Chem. 2011
Orthologous Events
Cite Us!