IVD

What is IVD Protein?

IVD protein, or isovaleryl-CoA dehydrogenase, is an enzyme involved in breaking down the amino acid leucine inside mitochondria. It helps turn leucine into energy. When the IVD protein malfunctions, it can cause isovaleric acidemia, a metabolic disorder. This condition might result in symptoms such as strange body odor and, in severe situations, neurological issues. Understanding how IVD operates is key to diagnosing and managing these metabolic challenges.

What is the Function of IVD Protein?

The IVD protein, known as isovaleryl-CoA dehydrogenase, is super important for breaking down the amino acid leucine in our mitochondria. Think of it like a key part of our body's power plant, helping turn what we eat into energy we need to move and think. When this protein doesn't work quite right, it can lead to a metabolic issue called Isovaleric Acidemia. This means certain toxic substances can build up, causing problems like feeling sick, really tired, or even having a unique "sweaty feet" smell. So, the IVD protein is all about making sure our body's engine runs smoothly by handling nutrients from our food.

IVD Related Signaling Pathway

The IVD protein doesn’t just hang out by itself; it’s linked to a bunch of different chemical signals in our cells that make up what’s called a signaling pathway. Basically, these pathways are like relay races. One molecule passes a “baton” to the next, and this protein is often a key player in pathways that manage energy production and metabolic processes. Sometimes, issues with these pathways can lead to problems in breaking down nutrients properly. For instance, it can impact things like fatty acid metabolism. When these pathways don’t run smoothly, it can disrupt energy balance in the body, leading to various metabolic disorders. So, IVD is pretty much a star in these pathways, ensuring everything from your energy levels to how efficiently your body uses nutrients, stays on track.

IVD Related Diseases

When IVD, or isovaleryl-CoA dehydrogenase, isn't doing its job right, it can cause a rare condition called Isovaleric Acidemia. This is basically a hiccup in the body's ability to break down leucine, one of those amino acids we get from food. Kids with this condition might suddenly start vomiting or seem really tired. One unusual sign is a sweaty feet smell, which is a tell-tale giveaway due to certain compounds building up. If caught early, with things like special diets or medications, folks can manage pretty well. But without attention, it can lead to more serious health issues. So, keeping an eye on how this protein works is key to avoiding or managing these kinds of metabolic bumps in the road.

Bioapplications of IVD

IVD protein is finding some cool uses in biotechnology. Beyond its critical role in metabolism, scientists are diving into how this protein helps us understand metabolic disorders like Isovaleric Acidemia. By studying IVD, they're working on better treatments that are tailored to the individual's needs. But that's not all—IVD is also making waves in the world of metabolic engineering. Researchers are tweaking it to improve processes like producing biofuels or other valuable substances using renewable resources. By figuring out and adjusting how IVD operates, they can create more efficient systems for these purposes. So, whether it's in medical research or sustainable industry, IVD is proving to be quite the versatile player.

Case Study 1: D'Annibale OM. et al. Mol Genet Metab. 2021

Newborn screening uses mass spectrometry to spot issues, but it often finds uncertain variants (VUS) that complicate diagnosing disorders like isovaleric acidemia (IVA). IVA stems from a lack of IVDH enzyme, with some mutations being unclear. Using CRISPR/Cas9, researchers made a cell model to test these VUS. Some mutations, such as p.Arg50Pro, showed reduced enzyme activity, while others like p.Val174Gly were normal. This method helps clarify VUS roles, aligning with IVA fibroblast findings.

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  Fig1. Western blot for IVDH confirms its absence in clonal lines.
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  Fig2. Detection of IVDH protein levels by western blot to evaluate protein stability.

Case Study 2: Millerioux Y. et al. PLoS Pathog. 2018

In Trypanosoma brucei, threonine, acetate, and glucose fuel both fatty acid and sterol production, while leucine is mostly for sterols in tsetse flies. Blocking key genes disrupts this balance, boosting fatty acid production. The parasites need ketogenic sources for growth, underscoring the mevalonate pathway's importance. Fatty acid synthesis is prioritized over sterols, with leucine as a primary sterol source in the fly’s gut.

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  Fig3. Western blot analysis of the EATRO1125.T7T, Δivdh-C3 and Δivdh-C8 cell lines.
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  Fig4. IVDH is needed for growth of PCF in medium depleted in ketogenic carbon sources.

IVD has several biochemical functions, for example, electron carrier activity,fatty-acyl-CoA binding,flavin adenine dinucleotide binding. Some of the functions are cooperated with other proteins, some of the functions could acted by IVD itself. We selected most functions IVD had, and list some proteins which have the same functions with IVD. You can find most of the proteins on our site.

Function	Related Protein
flavin adenine dinucleotide binding	ACAD11,FMO5,MAOB,NOX5,ACADSB,DLD,ACOX3,FMO1,SQLE,D2HGDH
electron carrier activity	GSR,TXNRD1,FDX1,NCF1,COX7A1,DERL3,ETFB,FDX1L,DHRS3,TSTA3
oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor	ACADVL,ACAD8,GCDH,ACOX2,ETFA,GCDHA,ACADL,ACOXL,ACADM,ACADSB
fatty-acyl-CoA binding	ACBD7,ACAD11,ALDH6A1,SCP2,ACBD5B,ECI2,ACADM,ACADL,GCDHB,HADHA

IVD has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with IVD here. Most of them are supplied by our site. Hope this information will be useful for your research of IVD.

ssuA;SIRT4