spastic paraplegia 7 (pure and complicated autosomal recessive), ELISA Kit

Human Paraplegin (SPG7) ELISA kit; CAR; CMAR; FLJ37308; MGC126331; MGC126332; PGN; SPG5C; cell adhesion regulator; cell matrix adhesion regulator; paraplegin; isoform 1; spastic paraplegia 7; spastic paraplegia 7 (pure and complicated autosomal recessive)

For Research Use Only. Not for use in diagnostic procedures.

No significant cross-reactivity or interference between this analyte and analogues is observed.

Store all reagents at 2-8 degree C

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Small volumes of SPG7 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9336618 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the spastic paraplegia 7 (pure and complicated autosomal recessive) (SPG7) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing SPG7. The ELISA analytical biochemical technique of the MBS9336618 kit is based on SPG7 antibody-SPG7 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect SPG7 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, SPG7. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Background: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determination the level of SPG7 (hereafter termed "analyte") in undiluted original Human body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances.

88,235 Da

spastic paraplegia 7 (pure and complicated autosomal recessive)

paraplegin; paraplegin, isoform 1; spastic paraplegia 7 protein; cell matrix adhesion regulator

Paraplegin

CAR; CMAR; PGN  [Similar Products]

SPG7_HUMAN

This gene encodes a mitochondrial metalloprotease protein that is a member of the AAA family. Members of this protein family share an ATPase domain and have roles in diverse cellular processes including membrane trafficking, intracellular motility, organelle biogenesis, protein folding, and proteolysis. Mutations in this gene cause autosomal recessive spastic paraplegia 7. Two transcript variants encoding distinct isoforms have been identified. [provided by RefSeq, Mar 2014]

SPG7: Putative ATP-dependent zinc metalloprotease. Defects in SPG7 are the cause of spastic paraplegia autosomal recessive type 7 (SPG7). Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. SPG7 is a complex form. Additional clinical features are cerebellar syndrome, supranuclear palsy, and cognitive impairment, particularly disturbance of attention and executive functions. Defects in SPG7 may cause autosomal recessive osteogenesis imperfecta (OI). Osteogenesis imperfecta defines a group of connective tissue disorders characterized by bone fragility and low bone mass. Clinical features of SPG7-related osteogenesis imperfecta include recurrent fractures, mild bone deformities, delayed tooth eruption, normal hearing and white sclera. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, multi-pass; Protease; Motility/polarity/chemotaxis; Cell adhesion; EC 3.4.24.-; Membrane protein, integral; Mitochondrial; Chaperone

Chromosomal Location of Human Ortholog: 16q24.3

Cellular Component: mitochondrion; mitochondrial membrane; integral to membrane

Molecular Function: peptidase activity; protein binding; zinc ion binding; metalloendopeptidase activity; unfolded protein binding; ATP binding

Biological Process: nervous system development; mitochondrion organization and biogenesis; proteolysis; anterograde axon cargo transport

Disease: Spastic Paraplegia 7, Autosomal Recessive

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