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Adrenoleukodystrophy – an X-linked, sex-linked,

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Presentation on theme: "Adrenoleukodystrophy – an X-linked, sex-linked,"— Presentation transcript:

1 Adrenoleukodystrophy – an X-linked, sex-linked,
recessive, genetic disorder Unaffected male Affected male Unaffected female Affected female Mr. Murphy Mrs. Murphy XAY XAXa Canise Mariah Michaela Augusto Diedre XAXa XAY XAXA XAY XAXa XAY XAX? XAXa Lorenzo Christina Francesco XAX? XAX? XAY XaY XAX? XAY

2 Augusto Adone’s Kitchen Sink Analogy for Long-Chain Saturated Fats
Tap = Diet as source of long-chain saturated fatty acids Sink = Lorenzo’s body Drain = enzyme which digests excess long-chain saturated fatty acids

3 Augusto Adone’s Kitchen Sink Analogy for Long-Chain Saturated Fats
Tap = Diet as source of long-chain saturated fatty acids Sink = Lorenzo’s body Plugged drain = missing enzyme in boys with ALD Drain = enzyme which digests excess long-chain saturated fatty acids

4 Augusto Adone’s Kitchen Sink Analogy for Long-Chain Saturated Fats
Tap = Diet as source of long-chain saturated fatty acids Rising water level in sink = Rising level of long-chain saturated fats in blood from diet, which destroy myelin Sink = Lorenzo’s body Plugged drain = missing enzyme in boys with ALD Drain = enzyme which digests excess long-chain saturated fatty acids

5 Augusto Adone’s Kitchen Sink Analogy for Long-Chain Saturated Fats
Tap = Diet as source of long-chain saturated fatty acids Tap turned off = therapy by diet, remove long-chain saturated fats from diet Rising water level in sink = Rising level of long-chain saturated fats in blood from diet, which destroy myelin Sink = Lorenzo’s body Plugged drain = missing enzyme in boys with ALD Drain = enzyme which digests excess long-chain saturated fatty acids

6 Augusto Adone’s Kitchen Sink Analogy for Long-Chain Saturated Fats
Tap turned on = increased biosynthesis maintaining high levels of long-chain saturated fats in blood Tap = Diet as source of long-chain saturated fatty acids Tap turned off = therapy by diet, remove long-chain saturated fats from diet Rising water level in sink = Rising level of long-chain saturated fats in blood from diet, which dissolve myelin Sink = Lorenzo’s body Plugged drain = missing enzyme in boys with ALD Drain = enzyme which digests excess long-chain saturated fatty acids

7 Long-Chain Saturated Fatty Acids
H H H H H H H H H H H H H H H H H O I I I I I I I I I I I I I I I I I II H—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—O I I I I I I I I I I I I I I I I I H H H H H H H H H H H H H H H H H H Saturated C18H36O2 Octadecanoic acid, or stearic acid H H H H H H H H H H H H H H H H H H H H H O I I I I I I I I I I I I I I I I I I I I I II H—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—O I I I I I I I I I I I I I I I I I I I I I H H H H H H H H H H H H H H H H H H H H H H Saturated C22H44O2 Docosanoic acid, or behenic acid H H H H H H H H H H H H H H H H H H H H H H H O I I I I I I I I I I I I I I I I I I I I I I I II H—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—C—O I I I I I I I I I I I I I I I I I I I I I I I H H H H H H H H H H H H H H H H H H H H H H H H Saturated C24H48O2 Tetracosanoic acid, or lignoceric acid

8 cis-9-Octadecenoic acid, or oleic acid
Long-Chain Monounsaturated Fatty Acids —C—C—C—C—C—C—C—C—O H H H H H H H O I I I I I I I II H H H H H H H H I I I I I I I H H H H H H H H I I I I I I I I H—C—C—C—C—C—C—C—C— I I I I I I I I H H H H H H H H — C — C H H Monounsaturated C18H34O2 cis-9-Octadecenoic acid, or oleic acid

9 cis-13-Docosenoic acid, or erucic acid
Long-Chain Monounsaturated Fatty Acids H H H H H H H H I I I I I I I I H—C—C—C—C—C—C—C—C— I I I I I I I I H H H H H H H H H —C—C—C—C—C—C—C—C—C—C—C—C—O H H H H H H H H H H H O I I I I I I I I I I I II H H H H H H H H H H H H I I I I I I I I I I I C — Monounsaturated C22 C42O2 cis-13-Docosenoic acid, or erucic acid

10 cis-15-Tetracosenoic acid, or nervonic acid
Long-Chain Monounsaturated Fatty Acids —C—C—C—C—C—C—C—C—C—C—C—C—C—C—O H H H H H H H H H H H H H O I I I I I I I I I I I I I II H H H H H H H H H H H H H H I I I I I I I I I I I I I H H H H H H H H I I I I I I I I H—C—C—C—C—C—C—C—C— I I I I I I I I H H H H H H H H — C — C H H Monounsaturated C24 C46O2 cis-15-Tetracosenoic acid, or nervonic acid

11 Competitive Inhibition – inhibiting biosynthesis of long-chain
saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

12 Competitive Inhibition – inhibiting biosynthesis of long-chain
saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

13 Competitive Inhibition – inhibiting biosynthesis of long-chain
saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid Diet therapy CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

14 Competitive Inhibition – inhibiting biosynthesis of long-chain
saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid Diet therapy CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

15 Competitive Inhibition – inhibiting biosynthesis of long-chain
saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid Diet therapy CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

16 Competitive Inhibition – inhibiting biosynthesis of long-chain
saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid Diet therapy Biosynthesis CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

17 Competitive Inhibition – inhibiting biosynthesis of long-chain
saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid C CH3(CH2)7CH CH(CH2)11COOH — Enzyme CH3(CH2)20COOH erucic acid behenic acid Diet therapy Biosynthesis CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

18 Competitive Inhibition – inhibiting biosynthesis of long-chain
saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid C CH3(CH2)7CH CH(CH2)11COOH — Enzyme CH3(CH2)20COOH erucic acid behenic acid Diet therapy Biosynthesis CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

19 Competitive Inhibition
Competitive Inhibition – inhibiting biosynthesis of long-chain saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid C Competitive Inhibition Enzyme CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid Diet therapy Biosynthesis CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

20 Competitive Inhibition
Competitive Inhibition – inhibiting biosynthesis of long-chain saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid C Competitive Inhibition Enzyme CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid Diet therapy Biosynthesis CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

21 Competitive Inhibition
Competitive Inhibition – inhibiting biosynthesis of long-chain saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid C Competitive Inhibition Enzyme CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid Diet therapy Biosynthesis CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

22 Competitive Inhibition
Competitive Inhibition – inhibiting biosynthesis of long-chain saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid C Competitive Inhibition Enzyme CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid C Diet therapy Biosynthesis CH3(CH2)7CH CH(CH2)13COOH — Enzyme CH3(CH2)22COOH nervonic acid lignoceric acid

23 Competitive Inhibition
Competitive Inhibition – inhibiting biosynthesis of long-chain saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid C Competitive Inhibition Enzyme CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid C Diet therapy Biosynthesis CH3(CH2)7CH CH(CH2)13COOH — Enzyme CH3(CH2)22COOH nervonic acid lignoceric acid

24 Competitive Inhibition Competitive Inhibition
Competitive Inhibition – inhibiting biosynthesis of long-chain saturated fatty acids by increasing biosynthesis of long-chain monounsaturated fatty acids CH3(CH2)7CH CH(CH2)7COOH — CH3(CH2)16COOH oleic acid stearic acid C Competitive Inhibition Enzyme CH3(CH2)7CH CH(CH2)11COOH — CH3(CH2)20COOH erucic acid behenic acid C Diet therapy Competitive Inhibition Enzyme CH3(CH2)7CH CH(CH2)13COOH — CH3(CH2)22COOH nervonic acid lignoceric acid

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