AFG3-like protein 2 (AFG3L2), ELISA Kit

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

Store all reagents at 2-8 degree C.

We suggest pre-experimenting with neat (undiluted) samples, 1:2 or 1:4 dilutions. Please avoid diluting your samples more than 1:10 as it would exceed the dilution limit set for this kit. If the expected concentration of the target is beyond the detection range of the kit, please contact our technical support team

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of AFG3L2 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.

MBS7230551 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the AFG3-like protein 2 (AFG3L2) 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 AFG3L2. The ELISA analytical biochemical technique of the MBS7230551 kit is based on AFG3L2 antibody-AFG3L2 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect AFG3L2 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, AFG3L2. 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(%).

88,584 Da

AFG3-like AAA ATPase 2

AFG3-like protein 2; paraplegin-like protein; ATPase family gene 3, yeast; AFG3 ATPase family gene 3-like 2; AFG3 ATPase family member 3-like 2

AFG3-like protein 2

AFG32_HUMAN

This gene encodes a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. This gene is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders. [provided by RefSeq, Jul 2008]

AFG3L2: ATP-dependent protease which is essential for axonal development. Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28). It is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA28 is an autosomal dominant cerebellar ataxia (ADCA) with a slow progressive course and no evidence of sensory involvement or cognitive impairment. Defects in AFG3L2 are the cause of spastic ataxia autosomal recessive type 5 (SPAX5). A neurodegenerative disorder characterized by early onset spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy.

Protein type: Membrane protein, integral; EC 3.4.24.-; Mitochondrial; Cell development/differentiation; Protease; Chaperone; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 18p11

Cellular Component: mitochondrion; mitochondrial inner membrane; integral to membrane

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

Biological Process: myelination; mitochondrial fusion; axonogenesis; cristae formation; righting reflex; death; regulation of multicellular organism growth; nerve development; proteolysis; muscle fiber development; neuromuscular junction development

Disease: Spinocerebellar Ataxia 28; Spastic Ataxia 5, Autosomal Recessive

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