Anti -Cblb, CT (Cblb, E3 ubiquitin-protein ligase CBL-B, Casitas B-lineage lymphoma proto-oncogene b, SH3-binding protein CBL-B, Signal transduction protein CBL-B)

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

Chromosome: 12; NC_000012.11 (109991520..110011358, complement). Location: 12q24

Polyclonal

IgG

Rabbit

Mouse

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-MMAB antibody 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.

E3 ubiquitin-protein ligase which accepts ubiquitin from specific E2 ubiquitin-conjugating enzymes, and transfers it to substrates, generally promoting their degradation by the proteasome. Negatively regulates TCR (T-cell receptor), BCR (B-cell receptor) and FCER1 (high affinity immunoglobulin epsilon receptor) signal transduction pathways. In naive T-cells, inhibits VAV1 activation upon TCR engagement and imposes a requirement for CD28 costimulation for proliferation and IL-2 production. Also acts by promoting PIK3R1/p85 ubiquitination, which impairs its recruitment to the TCR and subsequent activation. In activated T-cells, inhibits PLCG1 activation and calcium mobilization upon restimulation and promotes anergy. In B-cells, acts by ubiquitinating SYK and promoting its proteasomal degradation. May also be involved in EGFR ubiquitination and internalization.

Antibodies; Abs to Ubiquitin Ligases

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in Western Blot, ELISA
Dilution: ELISA: 1:1,000
Western Blot: 1:100-500

27,388 Da[Similar Products]

methylmalonic aciduria (cobalamin deficiency) cblB type

cob(I)yrinic acid a,c-diamide adenosyltransferase, mitochondrial; cob(I)yrinic acid a,c-diamide adenosyltransferase, mitochondrial; ATP:corrinoid adenosyltransferase; ATP:cob(I)alamin adenosyltransferase; methylmalonic aciduria type B protein; aquocob(I)alamin vitamin B12s adenosyltransferase

Cob(I)yrinic acid a,c-diamide adenosyltransferase, mitochondrial

MMAB_HUMAN

This gene encodes a protein that catalyzes the final step in the conversion of vitamin B(12) into adenosylcobalamin (AdoCbl), a vitamin B12-containing coenzyme for methylmalonyl-CoA mutase. Mutations in the gene are the cause of vitamin B12-dependent methylmalonic aciduria linked to the cblB complementation group. Alternatively spliced transcript variants have been found. [provided by RefSeq, Apr 2011]

MMAB: Defects in MMAB are the cause of methylmalonic aciduria type cblB (MMAB); also known as methylmalonic aciduria type B or vitamin B12-responsive methylmalonicaciduria of cblB complementation type. MMAB is a disorder of methylmalonate and cobalamin metabolism due to defective synthesis of adenosylcobalamin. Inheritance is autosomal recessive. Belongs to the Cob(I)alamin adenosyltransferase family.

Protein type: Transferase; Motility/polarity/chemotaxis; EC 2.5.1.17; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll

Chromosomal Location of Human Ortholog: 12q24

Cellular Component: mitochondrial matrix

Molecular Function: cob(I)yrinic acid a,c-diamide adenosyltransferase activity; ATP binding

Biological Process: vitamin metabolic process; cobalamin biosynthetic process; cobalamin metabolic process; water-soluble vitamin metabolic process

Disease: Methylmalonic Aciduria, Cblb Type

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