SPG21 Proteins, cDNA Clones Research Reagents

SPG21 (SPG21 Abhydrolase Domain Containing, Maspardin) is a protein coding gene located on human chromosome 15q22.31. SPG21 is also known as MAST, ACP33, GL010, ABHD21, and BM-019. The human SPG21 gene encodes a 34960 Da protein containing 308 amino acids. The SPG21 protein is ubiquitously expressed in thyroid, placenta and other tissues. Among its related pathways are Endocytosis. SPG21 is related to CD4 receptor binding. SPG21 is associated with some diseases, including Mast Syndrome and Paraplegia.

SPG21 Protein (2)

    SPG21 cDNA Clone (30)

    SPG21 Lysate (2)

      SPG21 Background

      Spastic paraplegia 21 (SPG21), also known as acid Cluster Protein 33 (ACP33) and Mast syndrome protein, is a member of the AB hydrolase superfamily. Human SPG21 is a 38 amino acid residue protein widely expressed in all tissues, including heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. SPG21 binds to the hydrophobic C-terminal amino acids of CD4 which are involved in repression of T cell activation via the noncatalytic alpha/beta hydrolase fold domain. SPG21 thus is proposed to play a role as a negative regulatory factor in CD4-dependent T-cell activation of CD4. Defects in SPG21 are the cause of spastic paraplegia autosomal recessive type 21, also known as Mast syndrome, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. SPG21 is also associated with dementia and other central nervous system abnormalities.

      SPG21 References

      • Zeitlmann L. et al., 2001, J Biol Chem. 276: 9123-32.
      • Simpson M. A. et al., 2003, Am J Hum Genet. 73: 1147-156.
      • Ota T. et al., 2004, Nat. Genet.36: 40-45.
      • Kedmi M. et al., 2007, Physiol Genomics. 28: 213-22.
      • Hanna M. C. et al., 2009, Neurogenetics.10: 217-28.

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