ATP-binding cassette sub-family B member 7, mitochondrial (ABCB7), 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

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Small volumes of ABCB7 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.

MBS7222910 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the ATP-binding cassette sub-family B member 7, mitochondrial (ABCB7) 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 ABCB7. The ELISA analytical biochemical technique of the MBS7222910 kit is based on ABCB7 antibody-ABCB7 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect ABCB7 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, ABCB7. 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(%).

Signal Transduction

78,160 Da

ATP-binding cassette, sub-family B (MDR/TAP), member 7

ATP-binding cassette sub-family B member 7, mitochondrial; ABC transporter 7 protein; ATP-binding cassette transporter 7

ATP-binding cassette sub-family B member 7, mitochondrial

ABC7; ABC transporter 7 protein  [Similar Products]

ABCB7_HUMAN

The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance as well as antigen presentation. This gene encodes a half-transporter involved in the transport of heme from the mitochondria to the cytosol. With iron/sulfur cluster precursors as its substrates, this protein may play a role in metal homeostasis. Mutations in this gene have been associated with mitochondrial iron accumulation and isodicentric (X)(q13) and sideroblastic anemia. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene. [provided by RefSeq, Nov 2012]

ABCB7: Could be involved in the transport of heme from the mitochondria to the cytosol. Plays a central role in the maturation of cytosolic iron-sulfur (Fe/S) cluster-containing proteins. Defects in ABCB7 are the cause of X-linked sideroblastic anemia with ataxia (ASAT). ASAT is a recessive disorder characterized by an infantile to early childhood onset of nonprogressive cerebellar ataxia and mild anemia with hypochromia and microcytosis. Belongs to the ABC transporter superfamily. ABCB family. Heavy Metal importer (TC 3.A.1.210) subfamily. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Transporter, ABC family; Transporter; Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial

Chromosomal Location of Human Ortholog: Xq13.3

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

Molecular Function: ATPase activity, coupled to transmembrane movement of substances; heme transporter activity; ATP binding

Biological Process: heme transport; cellular iron ion homeostasis; transport; transmembrane transport

Disease: Anemia, Sideroblastic, And Spinocerebellar Ataxia

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