SPG21 recombinant protein

Recombinant Human SPG21 Protein (GST tag)

Cat. Num. AAA2546204

• Gene Names: SPG21; MAST; ACP33; GL010; BM-019
• Purity: > 90 % as determined by SDS-PAGE
• Synonyms: SPG21; Recombinant Human SPG21 Protein (GST tag); ACP33; BM-019; GL010; MASPARDIN; MAST; SPG21 recombinant protein

• Host: Baculovirus-Insect Cells
• Purity/Purification: > 90 % as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile 50mM Tris, 100mM NaCl, pH 8.0, 10% glycerol
• Sequence Length: 308

• Application Notes: The recombinant human SPG21/GST chimera consists of 533 amino acids and predicts a molecular mass of 61 kDa which is also estimated by SDS-PAGE.
• Predicted N Terminal: Met
• Endotoxin: < 1.0 EU per mug of the protein as determined by the LAL method
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -70 degree C

SDS-PAGE

Related Product Information for SPG21 recombinant protein

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 308 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.

Description: A DNA sequence encoding the full length of human SPG21 (NP_057714.1) (Met 1-Gln 308) was expressed with the GST tag at the N-terminus.

NCBI and Uniprot Product Information

• NCBI GI #: 7706174
• NCBI GeneID: 51324
• NCBI Accession #: NP_057714.1
• NCBI GenBank Nucleotide #: NM_016630.6
• UniProt Accession #: Q9NZD8; Q6ZMB6; B4DW44
• Molecular Weight: 31,585 Da
• NCBI Official Full Name: maspardin isoform a
• NCBI Official Synonym Full Names: SPG21, maspardin
• NCBI Official Symbol: SPG21
• NCBI Official Synonym Symbols: MAST; ACP33; GL010; BM-019
• NCBI Protein Information: maspardin
• UniProt Protein Name: Maspardin
• Protein Family: Maspardin
• UniProt Gene Name: SPG21
• UniProt Synonym Gene Names: ACP33

NCBI Description

The protein encoded by this gene binds to the hydrophobic C-terminal amino acids of CD4 which are involved in repression of T cell activation. The interaction with CD4 is mediated by the noncatalytic alpha/beta hydrolase fold domain of this protein. It is thus proposed that this gene product modulates the stimulatory activity of CD4. Mutations in this gene are associated with autosomal recessive spastic paraplegia 21 (SPG21), also known as mast syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2014]

Uniprot Description

SPG21: May play a role as a negative regulatory factor in CD4- dependent T-cell activation. Defects in SPG21 are the cause of spastic paraplegia autosomal recessive type 21 (SPG21); also known as Mast syndrome. Spastic paraplegia is 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. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG21 is associated with dementia and other central nervous system abnormalities. Subtle childhood abnormalities may be present, but the main features develop in early adulthood. The disease is slowly progressive, and cerebellar and extrapyramidal signs are also found in patients with advanced disease. Patients have a thin corpus callosum and white-matter abnormalities. Belongs to the AB hydrolase superfamily. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Unknown function

Chromosomal Location of Human Ortholog: 15q22.31

Cellular Component: cytoplasm; cytosol; intracellular membrane-bound organelle; trans-Golgi network transport vesicle

Molecular Function: CD4 receptor binding; protein binding

Biological Process: antigen receptor-mediated signaling pathway

Disease: Mast Syndrome