AB96195

Anti-MMACHC/CblC antibody

Name: Anti-MMACHC/CblC antibody (ab96195) | Abcam
Brand: Abcam Limited
SKU: AB96195
Price: 480 USD
Availability: InStock

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(2 Publications)

Rabbit Polyclonal MMACHC/CblC antibody. Suitable for WB, ICC/IF, IHC-P and reacts with Mouse, Rat, Human samples. Cited in 2 publications. Immunogen corresponding to Recombinant Fragment Protein within Human MMACHC aa 1-200.

View Alternative Names

Cyanocobalamin reductase / alkylcobalamin dealkylase, Alkylcobalamin:glutathione S-alkyltransferase, CblC, Cyanocobalamin reductase (cyanide-eliminating), Methylmalonic aciduria and homocystinuria type C protein, MMACHC

5 Images

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMACHC/CblC antibody (AB96195)

IHC-P

Supplier Data

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMACHC/CblC antibody (AB96195)

Immunohistochemical analysis of paraffin-embedded c2c12 xenograft tissue with ab96195 at a 1/500 dilution.

Antigen retrieval : Trilogy™ (EDTA based, pH 8.0) buffer, 15min

IHC-P

Supplier Data

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMACHC/CblC antibody (AB96195)

Immunohistochemical analysis of paraffin-embedded human hepatoma tissue with ab96195 at a 1/500 dilution.

Antigen retrieval : Trilogy™ (EDTA based, pH 8.0) buffer, 15min.

Immunocytochemistry/ Immunofluorescence - Anti-MMACHC/CblC antibody (AB96195)

ICC/IF

Unknown

Immunocytochemistry/ Immunofluorescence - Anti-MMACHC/CblC antibody (AB96195)

ab96195, at 1/100 dilution, staining MMACHC/CblC in paraformaldehyde-fixed HeLa cells by Immunofluorescence. Lower image is merged with DNA probe.

Supplier Data

Western blot - Anti-MMACHC/CblC antibody (AB96195)

Samples were separated by 12% SDS-PAGE. The signal was developed with Trident ECL plus-Enhanced.

All lanes:

Western blot - Anti-MMACHC/CblC antibody (ab96195) at 1/1000 dilution

All lanes:

293T Whole cell extract at 30 µg

Secondary

All lanes:

HRP-conjugated anti-rabbit IgG antibody

Predicted band size: 32 kDa

true

Supplier Data

Western blot - Anti-MMACHC/CblC antibody (AB96195)

Samples were separated by 12% SDS-PAGE.

All lanes:

Western blot - Anti-MMACHC/CblC antibody (ab96195) at 1/500 dilution

Lane 1:

Mouse brain tissue extracts at 50 µg

Lane 2:

Rat brain tissue extracts at 50 µg

Secondary

All lanes:

HRP-conjugated anti-rabbit IgG antibody

Predicted band size: 32 kDa

false

Key facts

Host species

Rabbit

Clonality

Polyclonal

Isotype

IgG

Carrier free

Reacts with

Mouse, Rat, Human

Applications

ICC/IF, IHC-P, WB

applications

Immunogen

Recombinant Fragment Protein within Human MMACHC aa 1-200. The exact immunogen used to generate this antibody is proprietary information.

Q9Y4U1

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "ICCIF" : {"fullname" : "Immunocytochemistry/ Immunofluorescence", "shortname":"ICC/IF"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/500 - 1/3000", "WB-species-notes": "", "ICCIF-species-checked": "testedAndGuaranteed", "ICCIF-species-dilution-info": "1/100 - 1/1000", "ICCIF-species-notes": "", "IHCP-species-checked": "testedAndGuaranteed", "IHCP-species-dilution-info": "1/100 - 1/1000", "IHCP-species-notes": "" }, "Mouse": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/500 - 1/3000", "WB-species-notes": "", "ICCIF-species-checked": "guaranteed", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "IHCP-species-checked": "guaranteed", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" }, "Rat": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/500 - 1/3000", "WB-species-notes": "", "ICCIF-species-checked": "guaranteed", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "IHCP-species-checked": "guaranteed", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" }, "Cow": { "WB-species-checked": "predicted", "WB-species-dilution-info": "", "WB-species-notes": "", "ICCIF-species-checked": "predicted", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "IHCP-species-checked": "predicted", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" } } }

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Properties and storage information

Form

Liquid

Purification technique

Affinity purification Immunogen

Storage buffer

pH: 7 Preservative: 0.01% Thimerosal (merthiolate) Constituents: 10% Glycerol (glycerin, glycerine), 1.21% Tris, 0.75% Glycine

Shipped at conditions

Blue Ice

Appropriate short-term storage conditions

+4°C

Appropriate long-term storage conditions

-20°C

Aliquoting information

Upon delivery aliquot

Storage information

Avoid freeze / thaw cycle

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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

MMACHC also known as CblC is a protein involved in cobalamin (vitamin B12) metabolism. It acts as an intermediary in the processing of cobalamin facilitating its conversion into active derivatives needed for cellular reactions. MMACHC shows a molecular mass of around 31 kDa. This protein is mainly expressed in the cytosol of various tissues with high expression levels in the liver and brain. Proper functioning of MMACHC plays an important role in maintaining cellular metabolism and enzymatic reactions.

Biological function summary

MMACHC enables the conversion of cobalamin to its cofactor forms methylcobalamin and adenosylcobalamin. These forms are vital for key enzymatic reactions specifically in the methionine and methylmalonyl-CoA pathways. MMACHC functions as part of a larger cobalamin metabolic complex that also involves other critical enzymes and cofactors. This protein ensures proper activation of vitamin B12 essential for DNA synthesis and fatty acid metabolism.

Pathways

MMACHC is integral to both the methionine and methylmalonyl-CoA metabolic pathways. It interacts with methylmalonic acidemia proteins such as MUT and methionine synthase which are important for amino acid metabolism and red blood cell formation. These interactions coordinate the proper utilization of cobalamin influencing homocysteine levels and preventing its accumulation in the body which is significant for nerve function and cardiovascular health.

MMACHC mutations result in methylmalonic acidemia with homocystinuria a severe metabolic disorder. This condition highlights the malfunctioning of cobalamin metabolism leading to toxic accumulation of metabolites. MMACHC is also linked to certain neurodegenerative conditions due to its role in neuron health via proper vitamin B12 utilization. Researchers continue to study its complex interactions with other proteins including those in the transcobalamin complex to better understand how defects in its activity lead to disease manifestations.

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Product protocols

For this product, it's our understanding that no specific protocols are required. You can visit:

Visit the General protocols
Visit the Troubleshooting

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Target data

Cobalamin (vitamin B12) cytosolic chaperone that catalyzes the reductive decyanation of cyanocob(III)alamin (cyanocobalamin, CNCbl) to yield cob(II)alamin and cyanide, using FAD or FMN as cofactors and NADPH as cosubstrate (PubMed : 18779575, PubMed : 19700356, PubMed : 21697092, PubMed : 25809485). Cyanocobalamin constitutes the inactive form of vitamin B12 introduced from the diet, and is converted into the active cofactors methylcobalamin (MeCbl) involved in methionine biosynthesis, and 5'-deoxyadenosylcobalamin (AdoCbl) involved in the TCA cycle (PubMed : 19801555). Forms a complex with the lysosomal transporter ABCD4 and its chaperone LMBRD1, to transport cobalamin across the lysosomal membrane into the cytosol (PubMed : 25535791). The processing of cobalamin in the cytosol occurs in a multiprotein complex composed of at least MMACHC, MMADHC, MTRR (methionine synthase reductase) and MTR (methionine synthase) which may contribute to shuttle safely and efficiently cobalamin towards MTR in order to produce methionine (PubMed : 21071249, PubMed : 27771510). Also acts as a glutathione transferase by catalyzing the dealkylation of the alkylcob(III)alamins MeCbl and AdoCbl, using the thiolate of glutathione for nucleophilic displacement to generate cob(I)alamin and the corresponding glutathione thioether (PubMed : 19801555, PubMed : 21697092, PubMed : 22642810, PubMed : 25809485). The conversion of incoming MeCbl or AdoCbl into a common intermediate cob(I)alamin is necessary to meet the cellular needs for both cofactors (PubMed : 19801555). Cysteine and homocysteine cannot substitute for glutathione in this reaction (PubMed : 19801555).

See full target information MMACHC

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Publications (2)

Recent publications for all applications. Explore the full list and refine your search

American journal of human genetics 93:506-14 PubMed24011988

2013

An X-linked cobalamin disorder caused by mutations in transcriptional coregulator HCFC1.

Applications

Unspecified application

Species

Unspecified reactive species

Hung-Chun Yu,Jennifer L Sloan,Gunter Scharer,Alison Brebner,Anita M Quintana,Nathan P Achilly,Irini Manoli,Curtis R Coughlin,Elizabeth A Geiger,Una Schneck,David Watkins,Terttu Suormala,Johan L K Van Hove,Brian Fowler,Matthias R Baumgartner,David S Rosenblatt,Charles P Venditti,Tamim H Shaikh

Human molecular genetics 22:4591-601 PubMed23825108

2013

Interaction between methionine synthase isoforms and MMACHC: characterization in cblG-variant, cblG and cblC inherited causes of megaloblastic anaemia.

Applications

Unspecified application

Species

Unspecified reactive species

Ma'atem B Fofou-Caillierez,Nadir T Mrabet,Céline Chéry,Natacha Dreumont,Justine Flayac,Mihaela Pupavac,Justine Paoli,Jean-Marc Alberto,David Coelho,Jean-Michel Camadro,François Feillet,David Watkins,Brian Fowler,David S Rosenblatt,Jean-Louis Guéant

View all publications

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Product promise

We are committed to supporting your work with high-quality reagents, and we're here for you every step of the way. In the unlikely event that one of our products does not perform as expected, you're protected by our Product Promise.

For full details, please see our Terms & Conditions

Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.

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Anti-MMACHC/CblC antibody

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMACHC/CblC antibody (AB96195)

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-MMACHC/CblC antibody (AB96195)

Immunocytochemistry/ Immunofluorescence - Anti-MMACHC/CblC antibody (AB96195)

Western blot - Anti-MMACHC/CblC antibody (AB96195)

Western blot - Anti-MMACHC/CblC antibody (AB96195)

Key facts

Host species

Clonality

Isotype

Carrier free

Reacts with

Applications

Immunogen

Alternative Products

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Complementary Products

Primary Antibodies

Primary Antibodies

Primary Antibodies

Primary Antibodies

Reactivity data

Properties and storage information

Form

Purification technique

Storage buffer

Shipped at conditions

Appropriate short-term storage conditions

Appropriate long-term storage conditions

Aliquoting information

Storage information

Supplementary information

Biological function summary

Pathways

Product protocols

Target data

Publications (2)

Product promise