Acid Ceramidase Mouse, Unlabeled, Clone: 23, BD

Description

• Ceramide is a sphingolipid that exhibits a wide variety of functions, including monocyte differentiation, apoptosis, neurite outgrowth, and Ca2+ transport
• It also serves as the precursor of many sphingolipids and anchors these into the outer leaflet of the plasma membrane via hydrophobic interactions
• Acid ceramidase is a lysosomal enzyme that was purified from human urine
• It is synthesized as a 55kDa precursor protein, which is then processesed into the mature α-subunit (13kDa) and β-subunit (40kDa)
• Acid ceramidase catalyzes the hydrolysis of ceramide into free fatty acid and sphingosine
• Sphingosine, and its phosphorylated form, sphingosine-1-phosphate (SPP), have been shown to inhibit PKC activity and act as a second messenger in cell proliferation and differentiation
• Acid ceramidase is also the cause of a lysosomal storage disorder known as Farber’s disease
• This disease is characterized by an accumulation of ceramide in tissues, leading to swelling and pain of the joints and extremities, pulmonary insufficiency, and death at an early age
• Thus, acid ceramidase is necessary in the hydrolysis of ceramide and is the cause of Farber’s disease.