TY  - CHAP
M1  - Book, Section
TI  - Inborn Errors of Peroxisome Biogenesis and Function
A1  - Wanders, Ronald J.A.
A1  - Rizzo, William B.
A2  - Sarafoglou, Kyriakie
A2  - Hoffmann, Georg F.
A2  - Roth, Karl S.
PY  - 2017
T2  - Pediatric Endocrinology and Inborn Errors of Metabolism, 2e
AB  - The  essential  features  of  peroxisome  biogenesis  have  been  elucidated  in  recent  years.  These  features  are:  1)  peroxisomes  are  not  autonomously  multiplying  organelles  but  are  derived  from  the  endoplasmic  reticulum;  2)  all  peroxisomal  proteins,  including  peroxisomal  matrix  and  membrane  proteins,  are  encoded  by  the  nuclear  genome  and  synthesized  on  free  polyribosomes;  3)  the  newly  synthesized  proteins  are  post-translationally  imported  from  the  cytosol  into  preexisting  peroxisomes;  and  4)  import  of  new  peroxisomal  proteins  into  peroxisomes  leads  to  an  expansion  of  the  size  of  peroxisomes,  which  makes  them  grow  until  a  critical  size  is  reached.  Subsequently,  the  peroxisomes  divide  into  two  daughter  peroxisomes  that  can  then  undergo  the  same  cycle  of  events.  Figure  24-1  presents  a  simplified  scheme  of  peroxisome  biogenesis.  Correct  targeting  of  proteins  to  peroxisomes  is  achieved  via  so-called  peroxisome  targeting  signals  (PTSs).  Peroxisomal  matrix  proteins  are  targeted  to  peroxisomes  via  one  of  two  different  targeting  signals  which  are  short,  conserved  stretches  of  amino  acids  at  the  C-terminal  (PTS1)  or  N-terminal  (PTS2)  end  of  peroxisomal  matrix  proteins.  Several  relevant  proteins  that  possess  PTS1  or  PTS2  proteins  are  listed  in  Table  24-1.  Proteins  with  PTS  sequences  are  recognized  in  the  cytosol  by  receptor  proteins.  The  loaded  receptors  are  then  recognized  specifically  by  the  peroxisomal  protein  import  machinery,  after  which  the  matrix  proteins  are  translocated  across  the  peroxisomal  membrane,  whereas  the  receptors  are  released  back  into  the  cytosol  for  another  round  of  import.  In  principle,  peroxisomal  membrane  proteins  follow  a  similar  pathway,  although  the  targeting  signal  is  different  as  well  as  the  receptor  recognizing  this  signal  (Figure  24-1).  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1140318451
ER  -