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Acid Ceramidase Mouse, Unlabeled, Clone: 23, BD

Mouse Monoclonal Antibody

Manufacturer:  BD Biosciences 612302

Catalog No. BDB612302


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Description

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.

Specifications

Specifications

Acid Ceramidase
23
Unconjugated
Affinity Purified
Human Acid Ceramidase aa. 88-182
50μg
Cell Biology
Primary
Canine, Human
Western Blot
250μg/mL
Aqueous buffered solution containing BSA, glycerol, and ≤0.09% sodium azide.
Murine
IgG1
RUO
Store undiluted at -20°C.
Monoclonal
Documents
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