PMID- 16521125
OWN - NLM
STAT- MEDLINE
DCOM- 20060713
LR  - 20131121
IS  - 0360-4012 (Print)
IS  - 0360-4012 (Linking)
VI  - 83
IP  - 6
DP  - 2006 May 1
TI  - Influence of caloric restriction on motor behavior, longevity, and brain lipid 
      composition in Sandhoff disease mice.
PG  - 1028-38
AB  - Caloric restriction (CR), which improves health and increases longevity, was 
      studied as a therapy in a hexosaminidase beta knockout mouse model of Sandhoff 
      disease (SD), an incurable neurodegenerative disease involving accumulation of 
      brain ganglioside GM2 and asialo-GM2 (GA2). Adult mice were fed a rodent chow 
      diet either ad libitum (AL) or restricted to reduce body weight by 15-18% (CR). 
      Although GM2 and GA2 were elevated, no significant differences were seen between 
      the Hexb-/- and the Hexb+/- mice for most brain phospholipids and cholesterol. 
      Cerebrosides and sulfatides were reduced in the Hexb-/- mice. In addition, 
      rotorod performance was significantly worse in the Hexb-/- mice than in the 
      Hexb+/- mice. CR, which decreased circulating glucose and elevated ketone bodies, 
      significantly improved rotorod performance and extended longevity in the Hexb-/- 
      mice but had no significant effect on brain lipid composition or on cytoplasmic 
      neuronal vacuoles. The expression of CD68 and F4/80 was significantly less in the 
      CR-fed than in the AL-fed Hexb-/- mice. We suggest that the CR delays disease 
      progression in SD and possibly in other ganglioside storage diseases through 
      anti-inflammatory mechanisms.
CI  - 2006 Wiley-Liss, Inc.
FAU - Denny, Christine A
AU  - Denny CA
AD  - Department of Biology, Boston College, Chestnut Hill, Massachusetts 02467, USA.
FAU - Kasperzyk, Julie L
AU  - Kasperzyk JL
FAU - Gorham, Kristen N
AU  - Gorham KN
FAU - Bronson, Roderick T
AU  - Bronson RT
FAU - Seyfried, Thomas N
AU  - Seyfried TN
LA  - eng
GR  - HD39722/HD/NICHD NIH HHS/United States
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Neurosci Res
JT  - Journal of neuroscience research
JID - 7600111
RN  - 0 (Antigens, CD)
RN  - 0 (Antigens, Differentiation)
RN  - 0 (Antigens, Differentiation, Myelomonocytic)
RN  - 0 (Blood Glucose)
RN  - 0 (CD68 antigen, human)
RN  - 0 (Lipids)
RN  - 0 (RNA, Messenger)
RN  - 0 (monocyte-macrophage differentiation antigen)
RN  - EC 3.2.1.52 (Hexosaminidase B)
RN  - EC 3.2.1.52 (beta-N-Acetylhexosaminidases)
RN  - TZP1275679 (3-Hydroxybutyric Acid)
SB  - IM
MH  - 3-Hydroxybutyric Acid/blood
MH  - Age Factors
MH  - Animals
MH  - Antigens, CD/metabolism
MH  - Antigens, Differentiation/metabolism
MH  - Antigens, Differentiation, Myelomonocytic/metabolism
MH  - Behavior, Animal/physiology
MH  - Blood Glucose
MH  - Blotting, Western/methods
MH  - Body Weight/physiology
MH  - *Brain/metabolism/pathology/physiopathology
MH  - *Caloric Restriction
MH  - Cell Count/methods
MH  - Chromatography, High Pressure Liquid/methods
MH  - Disease Models, Animal
MH  - Hexosaminidase B
MH  - Immunohistochemistry/methods
MH  - *Lipids
MH  - Mice
MH  - Mice, Knockout
MH  - Motor Activity/*physiology
MH  - RNA, Messenger/metabolism
MH  - Reverse Transcriptase Polymerase Chain Reaction/methods
MH  - Rotarod Performance Test/methods
MH  - Sandhoff Disease/genetics/*metabolism/*physiopathology
MH  - beta-N-Acetylhexosaminidases/deficiency
EDAT- 2006/03/08 09:00
MHDA- 2006/07/14 09:00
CRDT- 2006/03/08 09:00
PHST- 2006/03/08 09:00 [pubmed]
PHST- 2006/07/14 09:00 [medline]
PHST- 2006/03/08 09:00 [entrez]
AID - 10.1002/jnr.20798 [doi]
PST - ppublish
SO  - J Neurosci Res. 2006 May 1;83(6):1028-38. doi: 10.1002/jnr.20798.