TY  - CHAP
M1  - Book, Section
TI  - Peroxisome Disorders
A1  - Wangler, Michael
A1  - Rizzo, William B.
A1  - Braverman, Nancy
A2  - Kline, Mark W.
PY  - 2018
T2  - Rudolph's Pediatrics, 23e
AB  - Peroxisomes  are  single  membrane-bound  organelles  present  in  virtually  all  eukaryotic  cells  that  number  between  hundreds  to  a  few  thousand  per  cell.  Peroxisomes  appear  on  electron  micrographs  as  spherical  organelles  (Fig.  157-1)  and  contain  a  dense  proteinaceous  matrix  composed  of  over  50  enzymes.  Peroxisomes  are  involved  in  a  diverse  list  of  metabolic  processes,  but  the  most  well  characterized  are  the  enzymatic  β-oxidation  of  very-long-chain  fatty  acids  and  other  substrates,  biosynthesis  of  plasmalogens  and  bile  acids,  α-oxidation  of  branched  chain  fatty  acids  (phytanic  acid),  and  glyoxylate  and  lysine  degradation.  Furthermore,  catalase  and  other  antioxidant  enzymes  in  peroxisomes  play  a  role  in  regulating  the  cellular  redox  balance.  Peroxisomes  form  through  the  concerted  action  of  an  evolutionarily  conserved  protein  machinery  encoded  by  the  PEX  genes  (Table  157-1).  The  PEX  gene  products  orchestrate  a  process  of  de  novo  peroxisome  biogenesis  that  begins  with  designation  of  membrane  compartment,  derived  from  the  endoplasmic  reticulum.  In  addition  to  de  novo  peroxisome  biogenesis,  peroxisomes  can  form  from  existing  peroxisomes  through  fission,  and  recent  studies  have  suggested  that  fission  may  play  at  least  as  important  a  role  in  creating  new  peroxisomes  as  de  novo  biogenesis.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/16
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1182929681
ER  -