Obesity is a cardinal aspect of the BBS phenotype, beginning in early childhood and
progressing with age; it is usually associated with the trunk and proximal limbs. A survey
of UK BBS patients identified 72% of adults as overweight (BMI 25) and 52%
defined as obese (BMI 30) . At present, the physiological and biochemical abnormalities
underlying obesity in BBS are poorly understood. A case-control study
showed no significant differences between resting metabolic rate between obese BBS
and controls suggesting no underlying defect in metabolism . Bbs-deficient mouse
models (Bbs4 and Bbs6) are initially runty at birth but display progressive weight gain
associated with increased food intake, culminating in obesity at 12 weeks
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